{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T05:53:42Z","timestamp":1790747622287,"version":"4.1.0"},"reference-count":180,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,9]]},"abstract":"\n OBJECTIVE<\/jats:title>\n Moyamoya angiopathy (MMA) affects the distal internal carotid artery and is designated as moyamoya disease (MMD) when predisposing conditions are absent, or moyamoya syndrome (MMS) when it occurs secondary to other causes. The authors aimed to investigate the reason for this anatomical site predilection of MMA. There is compelling evidence to suggest that MMA is a phenomenon that occurs due to stereotyped mechanobiological processes. Literature regarding MMD and MMS was systematically reviewed to decipher a common pattern relating to the development of MMA.<\/jats:p>\n <\/jats:sec>\n \n METHODS<\/jats:title>\n A systematic review was conducted to understand the pathogenesis of MMA in accordance with PRISMA guidelines. PubMed MEDLINE and Scopus were searched using \u201cmoyamoya\u201d and \u201cpathogenesis\u201d as common keywords and specific keywords related to six identified key factors. Additionally, a literature search was performed for MMS using \u201cmoyamoya\u201d and \u201cpathogenesis\u201d combined with reported associations. A progressive search of the literature was also performed using the keywords \u201cmatrix metalloprotease,\u201d \u201ctissue inhibitor of matrix metalloprotease,\u201d \u201cendothelial cell,\u201d \u201csmooth muscle cell,\u201d \u201ccytokines,\u201d \u201cendothelin,\u201d and \u201ctransforming growth factor\u201d to infer the missing links in molecular pathogenesis of MMA. Studies conforming to the inclusion criteria were reviewed.<\/jats:p>\n <\/jats:sec>\n \n RESULTS<\/jats:title>\n The literature search yielded 44 published articles on MMD by using keywords classified under the six key factors, namely arterial tortuosity, vascular angles, wall shear stress, molecular factors, blood rheology\/viscosity, and blood vessel wall strength, and 477 published articles on MMS associations. Information obtained from 51 articles that matched the inclusion criteria and additional information derived from the progressive search mentioned above were used to connect the key factors to derive a network pattern of pathogenesis.<\/jats:p>\n <\/jats:sec>\n \n CONCLUSIONS<\/jats:title>\n Based on the available literature, the authors have proposed a unifying theory for the pathogenesis of MMA. The moyamoya phenomenon appears to be the culmination of an interplay of vascular anatomy, hemodynamics, rheology, blood vessel wall strength, and a plethora of intricately linked mechanobiological molecular mediators that ultimately results in the mechanical process of occlusion of the blood vessel, stimulating angiogenesis and collateral blood supply in an attempt to perfuse the compromised brain.<\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/2021.6.focus21281","type":"journal-article","created":{"date-parts":[[2021,9,1]],"date-time":"2021-09-01T07:39:22Z","timestamp":1630481962000},"page":"E6","source":"Crossref","is-referenced-by-count":10,"title":["The mechanobiological theory: a unifying hypothesis on the pathogenesis of moyamoya disease based on a systematic review"],"prefix":"10.3171","volume":"51","author":[{"given":"Bhanu Jayanand","family":"Sudhir","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala State, India;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arun Gowda","family":"Keelara","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala State, India;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Easwer Harihara","family":"Venkat","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala State, India;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken","family":"Kazumata","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Hokkaido University Graduate School of Medicine, Sapporo, Japan; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ananthalakshmy","family":"Sundararaman","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Diseases and Diabetes Biology, Rajiv Gandhi Centre for Biotechnology, Trivandrum, Kerala State, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"7","key":"b1","series-title":"PLoS One","doi-asserted-by":"publisher","first-page":"e22542","DOI":"10.1371\/journal.pone.0022542","article-title":"Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development","volume":"6","author":"Liu W","year":"2011","unstructured":"Liu W, Morito D, Takashima S, Mineharu Y, Kobayashi H, Hitomi T, Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development. PLoS One. 2011;6(7):e22542.2179989210.1371\/journal.pone.0022542"},{"issue":"7","key":"b2","series-title":"PLoS Med","doi-asserted-by":"publisher","first-page":"e1000097","DOI":"10.1371\/journal.pmed.1000097","article-title":"Preferred Reporting Items for Systematic Reviews and Meta-Analyses: the PRISMA statement","volume":"6","author":"Moher D","year":"2009","unstructured":"Moher D, Liberati A, Tetzlaff J, Altman DG; PRISMA Group. Preferred Reporting Items for Systematic Reviews and Meta-Analyses: the PRISMA statement. PLoS Med. 2009;6(7):e1000097.10.1371\/journal.pmed.100009719621072"},{"key":"b3","series-title":"Thieme","article-title":"Pediatric Neurovascular Disease: Surgical, Endovascular, and Medical Management","author":"Alexander MJ","year":"2011","unstructured":"Alexander MJ, Spetzler RF. Pediatric Neurovascular Disease: Surgical, Endovascular, and Medical Management. Thieme;2011."},{"issue":"2","key":"b4","series-title":"J Stroke","doi-asserted-by":"publisher","first-page":"144","DOI":"10.5853\/jos.2015.17.2.144","article-title":"A functional perspective on the embryology and anatomy of the cerebral blood supply","volume":"17","author":"Menshawi K","year":"2015","unstructured":"Menshawi K, Mohr JP, Gutierrez J. A functional perspective on the embryology and anatomy of the cerebral blood supply. J Stroke. 2015;17(2):144\u2013158.10.5853\/jos.2015.17.2.14426060802"},{"issue":"5","key":"b5","series-title":"Acta Neurochir (Wien)","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1007\/s00701-015-2367-y","article-title":"Morphology and related hemodynamics of the internal carotid arteries of moyamoya patients","volume":"157","author":"Kim T","year":"2015","unstructured":"Kim T, Bang JS, Kwon OK, Hwang G, Kim JE, Kang HS, Morphology and related hemodynamics of the internal carotid arteries of moyamoya patients. Acta Neurochir (Wien). 2015;157(5):755\u2013761.10.1007\/s00701-015-2367-y"},{"issue":"16","key":"b6","series-title":"Comput Methods Biomech Biomed Engin","doi-asserted-by":"publisher","first-page":"1760","DOI":"10.1080\/10255842.2016.1184655","article-title":"Changes to the geometry and fluid mechanics of the carotid siphon in the pediatric moyamoya disease","volume":"19","author":"Jamil M","year":"2016","unstructured":"Jamil M, Tan GXY, Huq M, Kang H, Lee ZR, Tang PH, Changes to the geometry and fluid mechanics of the carotid siphon in the pediatric moyamoya disease. Comput Methods Biomech Biomed Engin. 2016;19(16):1760\u20131771.10.1080\/10255842.2016.1184655"},{"issue":"1","key":"b7","series-title":"Sci Rep","doi-asserted-by":"publisher","first-page":"8614","DOI":"10.1038\/s41598-019-45141-y","article-title":"Vascular tortuosity of the internal carotid artery is related to the RNF213 c.14429G > A variant in moyamoya disease","volume":"9","author":"An S","year":"2019","unstructured":"An S, Kim T, Oh CW, Bang JS, Lee SU, Heo J. Vascular tortuosity of the internal carotid artery is related to the RNF213 c.14429G > A variant in moyamoya disease. Sci Rep. 2019;9(1):8614.10.1038\/s41598-019-45141-y"},{"issue":"1","key":"b8","series-title":"Sci Rep","doi-asserted-by":"publisher","first-page":"16161","DOI":"10.1038\/srep16161","article-title":"Neuromuscular regulation in zebrafish by a large AAA+ ATPase\/ubiquitin ligase, mysterin\/RNF213","volume":"5","author":"Kotani Y","year":"2015","unstructured":"Kotani Y, Morito D, Yamazaki S, Ogino K, Kawakami K, Takashima S, Neuromuscular regulation in zebrafish by a large AAA+ ATPase\/ubiquitin ligase, mysterin\/RNF213. Sci Rep. 2015;5(1):16161.10.1038\/srep1616126530008"},{"issue":"18","key":"b9","series-title":"Neuroreport","doi-asserted-by":"publisher","first-page":"1442","DOI":"10.1097\/WNR.0000000000000289","article-title":"Increased vascular MMP-9 in mice lacking RNF213: moyamoya disease susceptibility gene","volume":"25","author":"Sonobe S","year":"2014","unstructured":"Sonobe S, Fujimura M, Niizuma K, Fujimura T, Furudate S, Nishijima Y, Increased vascular MMP-9 in mice lacking RNF213: moyamoya disease susceptibility gene. Neuroreport. 2014;25(18):1442\u20131446.2538346110.1097\/WNR.0000000000000289"},{"issue":"12","key":"b10","series-title":"Arq Neuropsiquiatr","doi-asserted-by":"publisher","first-page":"917","DOI":"10.1590\/S0004-282X2012001200003","article-title":"Carotid siphon geometry and variants of the circle of Willis in the origin of carotid aneurysms","volume":"70","author":"Silva Neto \u00c2R","year":"2012","unstructured":"Silva Neto \u00c2R, C\u00e2mara RLB, Valen\u00e7a MM. Carotid siphon geometry and variants of the circle of Willis in the origin of carotid aneurysms. Arq Neuropsiquiatr. 2012;70(12):917\u2013921.10.1590\/S0004-282X2012001200003"},{"issue":"9","key":"b11","series-title":"AJNR Am J Neuroradiol","doi-asserted-by":"publisher","first-page":"1771","DOI":"10.3174\/ajnr.A5285","article-title":"Influence of carotid siphon anatomy on brain aneurysm presentation","volume":"38","author":"Waihrich E","year":"2017","unstructured":"Waihrich E, Clavel P, Mendes GAC, Iosif C, Moraes Kessler I, Mounayer C. Influence of carotid siphon anatomy on brain aneurysm presentation. AJNR Am J Neuroradiol. 2017;38(9):1771\u20131775.10.3174\/ajnr.A528528684458"},{"key":"b12","first-page":"72","article-title":"Moyamoya disease: using computational fluid dynamics to propose a novel \u201cmechanical theory\u201d of pathogenesis","author":"Sudhir BJ","year":"2018","unstructured":"Sudhir BJ, Kumar KS. Moyamoya disease: using computational fluid dynamics to propose a novel \u201cmechanical theory\u201d of pathogenesis. In: 2018 International Conference on Intelligent Informatics and Biomedical Sciences (ICIIBMS).IEEE;2018:72\u201377."},{"issue":"12","key":"b13","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"2263","DOI":"10.1007\/s00381-013-2123-7","article-title":"Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery","volume":"29","author":"Lee JY","year":"2013","unstructured":"Lee JY, Kim SK, Cheon JE, Choi JW, Phi JH, Kim IO, Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery. Childs Nerv Syst. 2013;29(12):2263\u20132269.10.1007\/s00381-013-2123-723653141"},{"issue":"8","key":"b14","series-title":"PLoS One","doi-asserted-by":"publisher","first-page":"e0202526","DOI":"10.1371\/journal.pone.0202526","article-title":"Increased MMP activity in curved geometries disrupts the endothelial cell glycocalyx creating a proinflammatory environment","volume":"13","author":"Cooper S","year":"2018","unstructured":"Cooper S, Emmott A, McDonald KK, Campeau MA, Leask RL. Increased MMP activity in curved geometries disrupts the endothelial cell glycocalyx creating a proinflammatory environment. PLoS One. 2018;13(8):e0202526.3013840010.1371\/journal.pone.0202526"},{"issue":"3","key":"b15","series-title":"J Vasc Surg","doi-asserted-by":"publisher","first-page":"601","DOI":"10.1016\/j.jvs.2003.10.058","article-title":"Arterial enlargement, tortuosity, and intimal thickening in response to sequential exposure to high and low wall shear stress","volume":"39","author":"Sho E","year":"2004","unstructured":"Sho E, Nanjo H, Sho M, Kobayashi M, Komatsu M, Kawamura K, Arterial enlargement, tortuosity, and intimal thickening in response to sequential exposure to high and low wall shear stress. J Vasc Surg. 2004;39(3):601\u2013612.1498145510.1016\/j.jvs.2003.10.058"},{"issue":"2","key":"b16","series-title":"Exp Mol Pathol","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1006\/exmp.2002.2426","article-title":"Subnormal shear stress-induced intimal thickening requires medial smooth muscle cell proliferation and migration","volume":"72","author":"Sho M","year":"2002","unstructured":"Sho M, Sho E, Singh TM, Komatsu M, Sugita A, Xu C, Subnormal shear stress-induced intimal thickening requires medial smooth muscle cell proliferation and migration. Exp Mol Pathol. 2002;72(2):150\u2013160.10.1006\/exmp.2002.242611890724"},{"issue":"3","key":"b17","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"689","DOI":"10.3171\/2015.6.JNS15933","article-title":"Sonographic findings associated with stenosis progression and vascular complications in moyamoya disease","volume":"125","author":"Lee WJ","year":"2016","unstructured":"Lee WJ, Jung KH, Lee KJ, Kim JM, Lee ST, Chu K, Sonographic findings associated with stenosis progression and vascular complications in moyamoya disease. J Neurosurg. 2016;125(3):689\u2013697.10.3171\/2015.6.JNS1593326654185"},{"issue":"1","key":"b18","series-title":"Hellenic J Cardiol","first-page":"9","article-title":"Vascular wall shear stress: basic principles and methods","volume":"46","author":"Papaioannou TG","year":"2005","unstructured":"Papaioannou TG, Stefanadis C. Vascular wall shear stress: basic principles and methods. Hellenic J Cardiol. 2005;46(1):9\u201315.15807389"},{"issue":"8","key":"b19","series-title":"PLoS One","doi-asserted-by":"publisher","first-page":"e0161611","DOI":"10.1371\/journal.pone.0161611","article-title":"Time-series interactions of gene expression, vascular growth and hemodynamics during early embryonic arterial development","volume":"11","author":"Goktas S","year":"2016","unstructured":"Goktas S, Uslu FE, Kowalski WJ, Ermek E, Keller BB, Pekkan K. Time-series interactions of gene expression, vascular growth and hemodynamics during early embryonic arterial development. PLoS One. 2016;11(8):e0161611.2755215010.1371\/journal.pone.0161611"},{"issue":"1","key":"b20","series-title":"J Cell Physiol","doi-asserted-by":"publisher","first-page":"229","DOI":"10.1002\/jcp.20658","article-title":"Nitric oxide and p38 MAP kinase mediate shear stress-dependent inhibition of MMP-2 production in microvascular endothelial cells","volume":"208","author":"Milkiewicz M","year":"2006","unstructured":"Milkiewicz M, Kelland C, Colgan S, Haas TL. Nitric oxide and p38 MAP kinase mediate shear stress-dependent inhibition of MMP-2 production in microvascular endothelial cells. J Cell Physiol. 2006;208(1):229\u2013237.10.1002\/jcp.2065816575906"},{"issue":"8","key":"b21","series-title":"PLoS Comput Biol","doi-asserted-by":"publisher","first-page":"e1008161","DOI":"10.1371\/journal.pcbi.1008161","article-title":"Cell signaling model for arterial mechanobiology","volume":"16","author":"Irons L","year":"2020","unstructured":"Irons L, Humphrey JD. Cell signaling model for arterial mechanobiology. PLoS Comput Biol. 2020;16(8):e1008161.10.1371\/journal.pcbi.100816132834001"},{"issue":"5","key":"b22","series-title":"Surg Neurol","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1016\/j.surneu.2008.10.009","article-title":"Increased expression of serum matrix metalloproteinase-9 in patients with moyamoya disease","volume":"72","author":"Fujimura M","year":"2009","unstructured":"Fujimura M, Watanabe M, Narisawa A, Shimizu H, Tominaga T. Increased expression of serum matrix metalloproteinase-9 in patients with moyamoya disease. Surg Neurol. 2009;72(5):476\u2013480.1914719610.1016\/j.surneu.2008.10.009"},{"issue":"6","key":"b23","series-title":"J Neurol Neurosurg Psychiatry","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1136\/jnnp.2009.191817","article-title":"Plasma matrix metalloproteinases, cytokines and angiogenic factors in moyamoya disease","volume":"81","author":"Kang HS","year":"2010","unstructured":"Kang HS, Kim JH, Phi JH, Kim YY, Kim JE, Wang KC, Plasma matrix metalloproteinases, cytokines and angiogenic factors in moyamoya disease. J Neurol Neurosurg Psychiatry. 2010;81(6):673\u2013678.1996584410.1136\/jnnp.2009.191817"},{"issue":"6","key":"b24","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"1074","DOI":"10.1227\/01.NEU.0000215854.66011.4F","article-title":"Single nucleotide polymorphisms of tissue inhibitor of metalloproteinase genes in familial moyamoya disease","volume":"58","author":"Kang HS","year":"2006","unstructured":"Kang HS, Kim SK, Cho BK, Kim YY, Hwang YS, Wang KC. Single nucleotide polymorphisms of tissue inhibitor of metalloproteinase genes in familial moyamoya disease. Neurosurgery. 2006;58(6):1074\u20131080.10.1227\/01.NEU.0000215854.66011.4F16723886"},{"key":"b25","series-title":"BMC Neurol","doi-asserted-by":"publisher","first-page":"180","DOI":"10.1186\/s12883-014-0180-5","article-title":"The GC + CC genotype at position -418 in TIMP-2 promoter and the -1575GA\/-1306CC genotype in MMP-2 is genetic predisposing factors for prevalence of moyamoya disease","volume":"14","author":"Park YS","year":"2014","unstructured":"Park YS, Jeon YJ, Kim HS, Han IB, Oh SH, Kim DS, Kim NK. The GC + CC genotype at position -418 in TIMP-2 promoter and the -1575GA\/-1306CC genotype in MMP-2 is genetic predisposing factors for prevalence of moyamoya disease. BMC Neurol. 2014;14:180.10.1186\/s12883-014-0180-525280484"},{"key":"b26","series-title":"J Neurol Sci","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1016\/j.jns.2017.08.3380","article-title":"Concurrent moyamoya syndrome and Graves\u2019 disease: a case report","volume":"381","author":"Fu MH","year":"2017","unstructured":"Fu MH. Concurrent moyamoya syndrome and Graves\u2019 disease: a case report. J Neurol Sci. 2017;381:413.10.1016\/j.jns.2017.08.3380"},{"issue":"6","key":"b27","series-title":"J Stroke Cerebrovasc Dis","doi-asserted-by":"publisher","first-page":"528","DOI":"10.1016\/j.jstrokecerebrovasdis.2010.03.006","article-title":"Moyamoya syndrome associated with Graves\u2019 disease: a case report and review of the literature","volume":"20","author":"Malik S","year":"2011","unstructured":"Malik S, Russman AN, Katramados AM, Silver B, Mitsias PD. Moyamoya syndrome associated with Graves\u2019 disease: a case report and review of the literature. J Stroke Cerebrovasc Dis. 2011;20(6):528\u2013536.2113066810.1016\/j.jstrokecerebrovasdis.2010.03.006"},{"issue":"1","key":"b28","series-title":"Adv Clin Exp Med","doi-asserted-by":"publisher","first-page":"99","DOI":"10.17219\/acem\/68991","article-title":"Elevated serum concentrations of metalloproteinases (MMP-2, MMP-9) and their inhibitors (TIMP-1, TIMP-2) in patients with Graves\u2019 orbitopathy","volume":"27","author":"Kapelko-S\u0142owik K","year":"2018","unstructured":"Kapelko-S\u0142owik K, S\u0142owik M, Szali\u0144ski M, Dybko J, Wo\u0142owiec D, Prajs I, Elevated serum concentrations of metalloproteinases (MMP-2, MMP-9) and their inhibitors (TIMP-1, TIMP-2) in patients with Graves\u2019 orbitopathy. Adv Clin Exp Med. 2018;27(1):99\u2013103.10.17219\/acem\/6899129521049"},{"issue":"4","key":"b29","series-title":"Int J Radiat Oncol Biol Phys","doi-asserted-by":"publisher","first-page":"1222","DOI":"10.1016\/j.ijrobp.2006.01.038","article-title":"Radiation-induced moyamoya syndrome","volume":"65","author":"Desai SS","year":"2006","unstructured":"Desai SS, Paulino AC, Mai WY, Teh BS. Radiation-induced moyamoya syndrome. Int J Radiat Oncol Biol Phys. 2006;65(4):1222\u20131227.1662689010.1016\/j.ijrobp.2006.01.038"},{"issue":"6","key":"b30","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1159\/000446075","article-title":"Radiation-induced moyamoya syndrome after proton beam therapy in the pediatric patient: a case series","volume":"51","author":"Reynolds MR","year":"2016","unstructured":"Reynolds MR, Haydon DH, Caird J, Leonard JR. Radiation-induced moyamoya syndrome after proton beam therapy in the pediatric patient: a case series. Pediatr Neurosurg. 2016;51(6):297\u2013301.10.1159\/00044607527193755"},{"issue":"12","key":"b31","series-title":"Eur J Case Rep Intern Med","first-page":"001337","article-title":"Moyamoya syndrome after radiation therapy: a clinical report","volume":"6","author":"Almeida P","year":"2019","unstructured":"Almeida P, Rocha A, Alves G, Parreira T, Silva ML, Cerejo A, Moyamoya syndrome after radiation therapy: a clinical report. Eur J Case Rep Intern Med. 2019;6(12):001337.31893204"},{"issue":"5","key":"b32","series-title":"Int J Cancer","doi-asserted-by":"publisher","first-page":"766","DOI":"10.1002\/1097-0215(20001201)88:5<766::AID-IJC13>3.0.CO;2-Y","article-title":"Effects of radiation on the levels of MMP-2, MMP-9 and TIMP-1 during morphogenic glial-endothelial cell interactions","volume":"88","author":"Nirmala C","year":"2000","unstructured":"Nirmala C, Jasti SL, Sawaya R, Kyritsis AP, Konduri SD, Ali-Osman F, Effects of radiation on the levels of MMP-2, MMP-9 and TIMP-1 during morphogenic glial-endothelial cell interactions. Int J Cancer. 2000;88(5):766\u2013771.1107224610.1002\/1097-0215(20001201)88:5<766::AID-IJC13>3.0.CO;2-Y"},{"issue":"2","key":"b33","series-title":"Radiat Res","doi-asserted-by":"publisher","first-page":"174","DOI":"10.1667\/RR3107","article-title":"Retinoids and TIMP1 prevent radiation-induced apoptosis of capillary endothelial cells","volume":"161","author":"Vorotnikova E","year":"2004","unstructured":"Vorotnikova E, Tries M, Braunhut S. Retinoids and TIMP1 prevent radiation-induced apoptosis of capillary endothelial cells. Radiat Res. 2004;161(2):174\u2013184.1473107210.1667\/RR3107"},{"issue":"19","key":"b34","series-title":"Curr Med Chem","doi-asserted-by":"publisher","first-page":"2059","DOI":"10.2174\/092986710791233706","article-title":"Regulation of the protein C anticoagulant and antiinflammatory pathways","volume":"17","author":"Rezaie AR","year":"2010","unstructured":"Rezaie AR. Regulation of the protein C anticoagulant and antiinflammatory pathways. Curr Med Chem. 2010;17(19):2059\u20132069.10.2174\/09298671079123370620423310"},{"issue":"8","key":"b35","series-title":"Am J Physiol Cell Physiol","doi-asserted-by":"publisher","first-page":"C1109","DOI":"10.1152\/ajpcell.00369.2011","article-title":"Endothelial cells express a unique transcriptional profile under very high wall shear stress known to induce expansive arterial remodeling","volume":"302","author":"Dolan JM","year":"2012","unstructured":"Dolan JM, Sim FJ, Meng H, Kolega J. Endothelial cells express a unique transcriptional profile under very high wall shear stress known to induce expansive arterial remodeling. Am J Physiol Cell Physiol. 2012;302(8):C1109\u2013C1118.10.1152\/ajpcell.00369.201122173868"},{"issue":"suppl 1","key":"b36","series-title":"Saudi Med J","first-page":"S69","article-title":"Moyamoya syndrome as a risk factor for stroke in Saudi children Novel and usual associations","volume":"27","author":"Salih MA","year":"2006","unstructured":"Salih MA, Murshid WR, Al-Salman MM, Abdel-Gader AG, Al-Jarallah AA, Alorainy IA, Moyamoya syndrome as a risk factor for stroke in Saudi children Novel and usual associations. Saudi Med J. 2006;27(suppl 1):S69\u2013S80.16532134"},{"issue":"9","key":"b37","series-title":"Am J Hematol","doi-asserted-by":"publisher","first-page":"618","DOI":"10.1002\/ajh.21475","article-title":"Vasculopathy in sickle cell disease: biology, pathophysiology, genetics, translational medicine, and new research directions","volume":"84","author":"Kato GJ","year":"2009","unstructured":"Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: biology, pathophysiology, genetics, translational medicine, and new research directions. Am J Hematol. 2009;84(9):618\u2013625.10.1002\/ajh.2147519610078"},{"issue":"5","key":"b38","series-title":"Med Biol Eng Comput","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1007\/s11517-008-0330-2","article-title":"Wall shear stress as measured in vivo: consequences for the design of the arterial system","volume":"46","author":"Reneman RS","year":"2008","unstructured":"Reneman RS, Hoeks APG. Wall shear stress as measured in vivo: consequences for the design of the arterial system. Med Biol Eng Comput. 2008;46(5):499\u2013507.10.1007\/s11517-008-0330-218324431"},{"key":"b39","series-title":"Annu Rev Pathol","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1146\/annurev-pathmechdis-012418-012838","article-title":"Pathophysiology of sickle cell disease","volume":"14","author":"Sundd P","year":"2019","unstructured":"Sundd P, Gladwin MT, Novelli EM. Pathophysiology of sickle cell disease. Annu Rev Pathol. 2019;14:263\u2013292.3033256210.1146\/annurev-pathmechdis-012418-012838"},{"issue":"6","key":"b40","series-title":"Blood","doi-asserted-by":"publisher","first-page":"1584","DOI":"10.1182\/blood.V97.6.1584","article-title":"Endothelial dysfunction in patients with sickle cell disease is related to selective impairment of shear stress-mediated vasodilation","volume":"97","author":"Belhassen L","year":"2001","unstructured":"Belhassen L, Pelle G, Sediame S, Bachir D, Carville C, Bucherer C, Endothelial dysfunction in patients with sickle cell disease is related to selective impairment of shear stress-mediated vasodilation. Blood. 2001;97(6):1584\u20131589.10.1182\/blood.V97.6.158411238095"},{"issue":"11","key":"b41","series-title":"Blood","doi-asserted-by":"publisher","first-page":"1220","DOI":"10.1182\/blood.V108.11.1220.1220","article-title":"Increased levels and activities of matrix metalloproteinases in sickle cell disease","volume":"108","author":"Franco-Penteado CF","year":"2006","unstructured":"Franco-Penteado CF, Hyslop S, Conran N, Saad STO, Costa FF. Increased levels and activities of matrix metalloproteinases in sickle cell disease. Blood. 2006;108(11):1220.10.1182\/blood.V108.11.1220.1220"},{"issue":"12","key":"b42","series-title":"Circulation","doi-asserted-by":"publisher","first-page":"1530","DOI":"10.1161\/01.CIR.0000121741.50315.C2","article-title":"Increased carotid wall stress in vascular Ehlers-Danlos syndrome","volume":"109","author":"Boutouyrie P","year":"2004","unstructured":"Boutouyrie P, Germain DP, Fiessinger JN, Laloux B, Perdu J, Laurent S. Increased carotid wall stress in vascular Ehlers-Danlos syndrome. Circulation. 2004;109(12):1530\u20131535.10.1161\/01.CIR.0000121741.50315.C215007000"},{"issue":"6","key":"b43","series-title":"J Vasc Surg","doi-asserted-by":"publisher","first-page":"1263","DOI":"10.1067\/mva.2001.113579","article-title":"The role of type I collagen in aortic wall strength with a homotrimeric [\u03b11(I)]3 collagen mouse model","volume":"33","author":"Vouyouka AG","year":"2001","unstructured":"Vouyouka AG, Pfeiffer BJ, Liem TK, Taylor TA, Mudaliar J, Phillips CL. The role of type I collagen in aortic wall strength with a homotrimeric [\u03b11(I)]3 collagen mouse model. J Vasc Surg. 2001;33(6):1263\u20131270.10.1067\/mva.2001.11357911389427"},{"issue":"4","key":"b44","series-title":"AJNR Am J Neuroradiol","first-page":"748","article-title":"Abnormalities in the cerebral arterial system in osteogenesis imperfecta","volume":"24","author":"Albayram S","year":"2003","unstructured":"Albayram S, Kizilkilic O, Yilmaz H, Tuysuz B, Kocer N, Islak C. Abnormalities in the cerebral arterial system in osteogenesis imperfecta. AJNR Am J Neuroradiol. 2003;24(4):748\u2013750.12695216"},{"issue":"7","key":"b45","series-title":"Radiographics","doi-asserted-by":"publisher","first-page":"2101","DOI":"10.1148\/rg.327125716","article-title":"Craniospinal abnormalities and neurologic complications of osteogenesis imperfecta: imaging overview","volume":"32","author":"Khandanpour N","year":"2012","unstructured":"Khandanpour N, Connolly DJA, Raghavan A, Griffiths PD, Hoggard N. Craniospinal abnormalities and neurologic complications of osteogenesis imperfecta: imaging overview. Radiographics. 2012;32(7):2101\u20132112.2315086010.1148\/rg.327125716"},{"issue":"5","key":"b46","series-title":"Circ Res","doi-asserted-by":"publisher","first-page":"512","DOI":"10.1161\/CIRCRESAHA.107.157776","article-title":"Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in the thoracic aortic aneurysm in Marfan syndrome","volume":"101","author":"Chung AWY","year":"2007","unstructured":"Chung AWY, Au Yeung K, Sandor GGS, Judge DP, Dietz HC, van Breemen C. Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in the thoracic aortic aneurysm in Marfan syndrome. Circ Res. 2007;101(5):512\u2013522.10.1161\/CIRCRESAHA.107.15777617641224"},{"issue":"2","key":"b47","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"210","DOI":"10.1227\/NEU.0b013e31827b9beb","article-title":"Prevalence and characteristics of concurrent down syndrome in patients with moyamoya disease","volume":"72","author":"Kainth DS","year":"2013","unstructured":"Kainth DS, Chaudhry SA, Kainth HS, Suri FK, Qureshi AI. Prevalence and characteristics of concurrent down syndrome in patients with moyamoya disease. Neurosurgery. 2013;72(2):210\u2013215.2314996610.1227\/NEU.0b013e31827b9beb"},{"issue":"5","key":"b48","series-title":"Pediatrics","doi-asserted-by":"publisher","first-page":"e694","DOI":"10.1542\/peds.2005-0568","article-title":"Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization","volume":"116","author":"Jea A","year":"2005","unstructured":"Jea A, Smith ER, Robertson R, Scott RM. Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization. Pediatrics. 2005;116(5):e694\u2013e701.10.1542\/peds.2005-056816263984"},{"issue":"10","key":"b49","series-title":"FEBS J","doi-asserted-by":"publisher","first-page":"2418","DOI":"10.1111\/febs.12220","article-title":"New insights into the pathobiology of Down syndrome\u2014hyaluronan synthase-2 overexpression is regulated by collagen VI \u03b12 chain","volume":"280","author":"Karousou E","year":"2013","unstructured":"Karousou E, Stachtea X, Moretto P, Viola M, Vigetti D, D\u2019Angelo ML, New insights into the pathobiology of Down syndrome\u2014hyaluronan synthase-2 overexpression is regulated by collagen VI \u03b12 chain. FEBS J. 2013;280(10):2418\u20132430.10.1111\/febs.1222023452080"},{"issue":"34","key":"b50","series-title":"Proc Natl Acad Sci U S A","doi-asserted-by":"publisher","first-page":"20753","DOI":"10.1073\/pnas.2004281117","article-title":"Identifying collagen VI as a target of fibrotic diseases regulated by CREBBP\/EP300","volume":"117","author":"Williams LM","year":"2020","unstructured":"Williams LM, McCann FE, Cabrita MA, Layton T, Cribbs A, Knezevic B, Identifying collagen VI as a target of fibrotic diseases regulated by CREBBP\/EP300. Proc Natl Acad Sci U S A. 2020;117(34):20753\u201320763.10.1073\/pnas.2004281117"},{"issue":"11","key":"b51","series-title":"Biochim Biophys Acta","doi-asserted-by":"publisher","first-page":"2106","DOI":"10.1016\/j.bbadis.2014.07.008","article-title":"Vascular wall extracellular matrix proteins and vascular diseases","volume":"1842","author":"Xu J","year":"2014","unstructured":"Xu J, Shi GP. Vascular wall extracellular matrix proteins and vascular diseases. Biochim Biophys Acta. 2014;1842(11):2106\u20132119.2504585410.1016\/j.bbadis.2014.07.008"},{"key":"b52","series-title":"Vasc Health Risk Manag","doi-asserted-by":"publisher","first-page":"375","DOI":"10.2147\/VHRM.S21273","article-title":"Stiffness of the large arteries in individuals with and without Down syndrome","volume":"7","author":"Rodrigues AN","year":"2011","unstructured":"Rodrigues AN, Coelho LC, Goncalves WL, Gouvea SA, Vasconcellos MJ, Cunha RS, Abreu GR. Stiffness of the large arteries in individuals with and without Down syndrome. Vasc Health Risk Manag. 2011;7:375\u2013381.10.2147\/VHRM.S2127321731889"},{"issue":"2","key":"b53","series-title":"J Ment Defic Res","first-page":"123","article-title":"Blood pressure in Down\u2019s syndrome","volume":"23","author":"Richards BW","year":"1979","unstructured":"Richards BW, Enver F. Blood pressure in Down\u2019s syndrome. J Ment Defic Res. 1979;23(2):123\u2013135.158658"},{"issue":"4","key":"b54","series-title":"Pediatrics","doi-asserted-by":"publisher","first-page":"e20180840","DOI":"10.1542\/peds.2018-0840","article-title":"Blood pressure elevation and risk of moyamoya syndrome in patients with trisomy 21","volume":"142","author":"Santoro JD","year":"2018","unstructured":"Santoro JD, Lee S, Mlynash M, Nguyen T, Lazzareschi DV, Kraler LD, Blood pressure elevation and risk of moyamoya syndrome in patients with trisomy 21. Pediatrics. 2018;142(4):e20180840.3019034710.1542\/peds.2018-0840"},{"issue":"1","key":"b55","series-title":"Neurobiol Dis","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1006\/nbdi.1995.0002","article-title":"Expression of the neurofibromatosis I gene product, neurofibromin, in blood vessel endothelial cells and smooth muscle","volume":"2","author":"Norton KK","year":"1995","unstructured":"Norton KK, Xu J, Gutmann DH. Expression of the neurofibromatosis I gene product, neurofibromin, in blood vessel endothelial cells and smooth muscle. Neurobiol Dis. 1995;2(1):13\u201321.10.1006\/nbdi.1995.00028980005"},{"issue":"3","key":"b56","series-title":"Genet Med","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1097\/00125817-200205000-00002","article-title":"Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force","volume":"4","author":"Friedman JM","year":"2002","unstructured":"Friedman JM, Arbiser J, Epstein JA, Gutmann DH, Huot SJ, Lin AE, Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force. Genet Med. 2002;4(3):105\u2013111.1218014310.1097\/00125817-200205000-00002"},{"key":"b57","article-title":"MedlinePlus Genetics","author":"HRAS gene","unstructured":"HRAS gene. MedlinePlus Genetics. Accessed July 12, 2021.https:\/\/medlineplus.gov\/genetics\/gene\/hras\/"},{"issue":"4","key":"b58","series-title":"Arterioscler Thromb Vasc Biol","doi-asserted-by":"publisher","first-page":"582","DOI":"10.1161\/01.ATV.0000064372.78561.A5","article-title":"Elastogenesis in human arterial disease: a role for macrophages in disordered elastin synthesis","volume":"23","author":"Krettek A","year":"2003","unstructured":"Krettek A, Sukhova GK, Libby P. Elastogenesis in human arterial disease: a role for macrophages in disordered elastin synthesis. Arterioscler Thromb Vasc Biol. 2003;23(4):582\u2013587.1261567410.1161\/01.ATV.0000064372.78561.A5"},{"issue":"5","key":"b59","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1016\/j.pediatrneurol.2004.12.010","article-title":"Costello syndrome showing moyamoya-like vasculopathy","volume":"32","author":"Shiihara T","year":"2005","unstructured":"Shiihara T, Kato M, Mitsuhashi Y, Hayasaka K. Costello syndrome showing moyamoya-like vasculopathy. Pediatr Neurol. 2005;32(5):361\u2013363.10.1016\/j.pediatrneurol.2004.12.01015866441"},{"issue":"4","key":"b60","series-title":"Dermatology","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1159\/000051558","article-title":"Costello syndrome with decreased gene expression of elastin in cultured dermal fibroblasts","volume":"201","author":"Hatamochi A","year":"2000","unstructured":"Hatamochi A, Nagayama H, Kuroda K, Shinkai H, Ishikiriyama S, Kobayashi M, Kobayashi K. Costello syndrome with decreased gene expression of elastin in cultured dermal fibroblasts. Dermatology. 2000;201(4):366\u2013369.10.1159\/00005155811146354"},{"issue":"3","key":"b61","series-title":"Curr Opin Genet Dev","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1016\/j.gde.2009.04.001","article-title":"The RASopathies: developmental syndromes of Ras\/MAPK pathway dysregulation","volume":"19","author":"Tidyman WE","year":"2009","unstructured":"Tidyman WE, Rauen KA. The RASopathies: developmental syndromes of Ras\/MAPK pathway dysregulation. Curr Opin Genet Dev. 2009;19(3):230\u2013236.1946785510.1016\/j.gde.2009.04.001"},{"issue":"2","key":"b62","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1016\/j.pediatrneurol.2011.03.007","article-title":"Moyamoya syndrome in a child with Noonan syndrome","volume":"45","author":"Hung PC","year":"2011","unstructured":"Hung PC, Wang HS, Wong AMC. Moyamoya syndrome in a child with Noonan syndrome. Pediatr Neurol. 2011;45(2):129\u2013131.10.1016\/j.pediatrneurol.2011.03.007"},{"issue":"4","key":"b63","series-title":"Brain Dev","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/S0387-7604(02)00014-1","article-title":"Cardio-facio-cutaneous syndrome and moyamoya syndrome","volume":"24","author":"Ishiguro Y","year":"2002","unstructured":"Ishiguro Y, Kubota T, Takenaka J, Maruyama K, Okumura A, Negoro T, Watanabe K. Cardio-facio-cutaneous syndrome and moyamoya syndrome. Brain Dev. 2002;24(4):245\u2013249.10.1016\/S0387-7604(02)00014-112015168"},{"issue":"1","key":"b64","series-title":"Am J Med Genet A","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1002\/ajmg.a.61921","article-title":"Moyamoya syndrome in a child with Legius syndrome: Introducing a cerebral vasculopathy to the SPRED1 phenotype?","volume":"185","author":"Pabst L","year":"2021","unstructured":"Pabst L, Carroll J, Lo W, Truxal KV. Moyamoya syndrome in a child with Legius syndrome: Introducing a cerebral vasculopathy to the SPRED1 phenotype?. Am J Med Genet A. 2021;185(1):223\u2013227.10.1002\/ajmg.a.61921"},{"issue":"2","key":"b65","series-title":"Pediatrics","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1542\/peds.103.2.505","article-title":"Moyamoya syndrome in children with Alagille syndrome: additional evidence of a vasculopathy","volume":"103","author":"Woolfenden AR","year":"1999","unstructured":"Woolfenden AR, Albers GW, Steinberg GK, Hahn JS, Johnston DC, Farrell K. Moyamoya syndrome in children with Alagille syndrome: additional evidence of a vasculopathy. Pediatrics. 1999;103(2):505\u2013508.992585310.1542\/peds.103.2.505"},{"issue":"1","key":"b66","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1159\/000204907","article-title":"Identification of an association between Robinow syndrome and moyamoya","volume":"45","author":"Qaiser R","year":"2009","unstructured":"Qaiser R, Scott RM, Smith ER. Identification of an association between Robinow syndrome and moyamoya. Pediatr Neurosurg. 2009;45(1):69\u201372.1925873310.1159\/000204907"},{"key":"b67","series-title":"Int J Vasc Med","first-page":"293641","article-title":"Signaling required for blood vessel maintenance: molecular basis and pathological manifestations","volume":"2012","author":"Murakami M","year":"2012","unstructured":"Murakami M. Signaling required for blood vessel maintenance: molecular basis and pathological manifestations. Int J Vasc Med. 2012;2012:293641.22187650"},{"issue":"1","key":"b68","series-title":"Dev Cell","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/j.devcel.2008.12.009","article-title":"Nrarp coordinates endothelial Notch and Wnt signaling to control vessel density in angiogenesis","volume":"16","author":"Phng LK","year":"2009","unstructured":"Phng LK, Potente M, Leslie JD, Babbage J, Nyqvist D, Lobov I, Nrarp coordinates endothelial Notch and Wnt signaling to control vessel density in angiogenesis. Dev Cell. 2009;16(1):70\u201382.1915471910.1016\/j.devcel.2008.12.009"},{"issue":"2","key":"b69","series-title":"J Med Genet","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1136\/jmg.2006.044313","article-title":"Schimke immuno-osseous dysplasia: a clinicopathological correlation","volume":"44","author":"Clewing JM","year":"2007","unstructured":"Clewing JM, Antalfy BC, L\u00fccke T, Najafian B, Marwedel KM, Hori A, Schimke immuno-osseous dysplasia: a clinicopathological correlation. J Med Genet. 2007;44(2):122\u2013130.1684056810.1136\/jmg.2006.044313"},{"issue":"10","key":"b70","series-title":"Eur J Pediatr","doi-asserted-by":"publisher","first-page":"1567","DOI":"10.1007\/s00431-012-1732-6","article-title":"Medical management of moyamoya disease and recurrent stroke in an infant with Majewski osteodysplastic primordial dwarfism type II (MOPD II)","volume":"171","author":"K\u0131l\u0131\u00e7 E","year":"2012","unstructured":"K\u0131l\u0131\u00e7 E, Utine E, \u00dcnal S, Halilo\u011flu G, O\u011fuz KK, Cetin M, Medical management of moyamoya disease and recurrent stroke in an infant with Majewski osteodysplastic primordial dwarfism type II (MOPD II). Eur J Pediatr. 2012;171(10):1567\u20131571.10.1007\/s00431-012-1732-6"},{"issue":"4","key":"b71","series-title":"J Neurosurg Pediatr","doi-asserted-by":"publisher","first-page":"320","DOI":"10.3171\/2008.12.PEDS08205","article-title":"Seckel syndrome and moyamoya","volume":"3","author":"Codd PJ","year":"2009","unstructured":"Codd PJ, Scott RM, Smith ER. Seckel syndrome and moyamoya. J Neurosurg Pediatr. 2009;3(4):320\u2013324.1933841210.3171\/2008.12.PEDS08205"},{"key":"b72","series-title":"Appl Clin Genet","first-page":"49","article-title":"Moyamoya disease and syndromes: from genetics to clinical management","volume":"8","author":"Guey S","year":"2015","unstructured":"Guey S, Tournier-Lasserve E, Herv\u00e9 D, Kossorotoff M. Moyamoya disease and syndromes: from genetics to clinical management. Appl Clin Genet. 2015;8:49\u201368.25733922"},{"issue":"6","key":"b73","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"718","DOI":"10.1016\/j.ajhg.2011.04.017","article-title":"Loss of BRCC3 deubiquitinating enzyme leads to abnormal angiogenesis and is associated with syndromic moyamoya","volume":"88","author":"Miskinyte S","year":"2011","unstructured":"Miskinyte S, Butler MG, Herv\u00e9 D, Sarret C, Nicolino M, Petralia JD, Loss of BRCC3 deubiquitinating enzyme leads to abnormal angiogenesis and is associated with syndromic moyamoya. Am J Hum Genet. 2011;88(6):718\u2013728.10.1016\/j.ajhg.2011.04.01721596366"},{"issue":"1","key":"b74","series-title":"Int J Mol Med","first-page":"29","article-title":"Expression of BRCC3, a novel cell cycle regulated molecule, is associated with increased phospho-ERK and cell proliferation","volume":"19","author":"Boudreau HE","year":"2007","unstructured":"Boudreau HE, Broustas CG, Gokhale PC, Kumar D, Mewani RR, Rone JD, Expression of BRCC3, a novel cell cycle regulated molecule, is associated with increased phospho-ERK and cell proliferation. Int J Mol Med. 2007;19(1):29\u201339.17143545"},{"issue":"10","key":"b75","series-title":"Neurol Med Chir (Tokyo)","doi-asserted-by":"publisher","first-page":"361","DOI":"10.2176\/nmc.ra.2019-0059","article-title":"Influence of inflammatory disease on the pathophysiology of moyamoya disease and quasi-moyamoya disease","volume":"59","author":"Mikami T","year":"2019","unstructured":"Mikami T, Suzuki H, Komatsu K, Mikuni N. Influence of inflammatory disease on the pathophysiology of moyamoya disease and quasi-moyamoya disease. Neurol Med Chir (Tokyo). 2019;59(10):361\u2013370.10.2176\/nmc.ra.2019-0059"},{"issue":"9","key":"b76","series-title":"J Infect Dis","doi-asserted-by":"publisher","first-page":"1411","DOI":"10.1086\/374644","article-title":"Matrix metalloproteinase-9 in pneumococcal meningitis: activation via an oxidative pathway","volume":"187","author":"Meli DN","year":"2003","unstructured":"Meli DN, Christen S, Leib SL. Matrix metalloproteinase-9 in pneumococcal meningitis: activation via an oxidative pathway. J Infect Dis. 2003;187(9):1411\u20131415.10.1086\/37464412717622"},{"issue":"1","key":"b77","series-title":"BMC Infect Dis","doi-asserted-by":"publisher","first-page":"722","DOI":"10.1186\/s12879-016-1953-9","article-title":"Title: role of matrix metalloproteinase -9 in progression of tuberculous meningitis: a pilot study in patients at different stages of the disease","volume":"16","author":"Majeed S","year":"2016","unstructured":"Majeed S, Singh P, Sharma N, Sharma S. Title: role of matrix metalloproteinase -9 in progression of tuberculous meningitis: a pilot study in patients at different stages of the disease. BMC Infect Dis. 2016;16(1):722.10.1186\/s12879-016-1953-927899068"},{"key":"b78","series-title":"Clin Neurol Neurosurg","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1016\/j.clineuro.2014.12.001","article-title":"Moyamoya in a patient with Sneddon\u2019s syndrome","volume":"129","author":"Fierini F","year":"2015","unstructured":"Fierini F, Barilaro A, Giambene B, Carlucci G, Grandi V, Maio V, Pantoni L. Moyamoya in a patient with Sneddon\u2019s syndrome. Clin Neurol Neurosurg. 2015;129:34\u201336.10.1016\/j.clineuro.2014.12.001"},{"issue":"10","key":"b79","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1056\/NEJMoa1307361","article-title":"Early-onset stroke and vasculopathy associated with mutations in ADA2","volume":"370","author":"Zhou Q","year":"2014","unstructured":"Zhou Q, Yang D, Ombrello AK, Zavialov AV, Toro C, Zavialov AV, Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med. 2014;370(10):911\u2013920.10.1056\/NEJMoa130736124552284"},{"issue":"2","key":"b80","series-title":"J Stroke Cerebrovasc Dis","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/S1052-3057(99)80054-8","article-title":"Moyamoya syndrome associated with Sneddon\u2019s syndrome and antiphospholipid-protein antibodies","volume":"8","author":"Carhuapoma JR","year":"1999","unstructured":"Carhuapoma JR, D\u2019Olhaberriague L, Levine SR. Moyamoya syndrome associated with Sneddon\u2019s syndrome and antiphospholipid-protein antibodies. J Stroke Cerebrovasc Dis. 1999;8(2):51\u201356.10.1016\/S1052-3057(99)80054-817895140"},{"issue":"8","key":"b81","series-title":"Dev Med Child Neurol","doi-asserted-by":"publisher","first-page":"725","DOI":"10.1111\/j.1469-8749.2010.03727.x","article-title":"Intracerebral large artery disease in Aicardi-Gouti\u00e8res syndrome implicates SAMHD1 in vascular homeostasis","volume":"52","author":"Ramesh V","year":"2010","unstructured":"Ramesh V, Bernardi B, Stafa A, Garone C, Franzoni E, Abinun M, Intracerebral large artery disease in Aicardi-Gouti\u00e8res syndrome implicates SAMHD1 in vascular homeostasis. Dev Med Child Neurol. 2010;52(8):725\u2013732.2065373610.1111\/j.1469-8749.2010.03727.x"},{"issue":"7","key":"b82","series-title":"Nat Rev Immunol","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1038\/nri3850","article-title":"Aicardi-Gouti\u00e8res syndrome and the type I interferonopathies","volume":"15","author":"Crow YJ","year":"2015","unstructured":"Crow YJ, Manel N. Aicardi-Gouti\u00e8res syndrome and the type I interferonopathies. Nat Rev Immunol. 2015;15(7):429\u2013440.10.1038\/nri385026052098"},{"issue":"3","key":"b83","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1016\/j.ajhg.2014.01.018","article-title":"Loss of \u03b11\u03b21 soluble guanylate cyclase, the major nitric oxide receptor, leads to moyamoya and achalasia","volume":"94","author":"Herv\u00e9 D","year":"2014","unstructured":"Herv\u00e9 D, Philippi A, Belbouab R, Zerah M, Chabrier S, Collardeau-Frachon S, Loss of \u03b11\u03b21 soluble guanylate cyclase, the major nitric oxide receptor, leads to moyamoya and achalasia. Am J Hum Genet. 2014;94(3):385\u2013394.2458174210.1016\/j.ajhg.2014.01.018"},{"issue":"5","key":"b84","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"617","DOI":"10.1016\/j.ajhg.2009.04.007","article-title":"Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and moyamoya disease, along with thoracic aortic disease","volume":"84","author":"Guo DC","year":"2009","unstructured":"Guo DC, Papke CL, Tran-Fadulu V, Regalado ES, Avidan N, Johnson RJ, Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and moyamoya disease, along with thoracic aortic disease. Am J Hum Genet. 2009;84(5):617\u2013627.1940952510.1016\/j.ajhg.2009.04.007"},{"issue":"2","key":"b85","series-title":"Stroke","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1161\/01.STR.22.2.269","article-title":"Turner\u2019s syndrome, fibromuscular dysplasia, and stroke","volume":"22","author":"Lancman M","year":"1991","unstructured":"Lancman M, Mesropian H, Serra P, Granillo R. Turner\u2019s syndrome, fibromuscular dysplasia, and stroke. Stroke. 1991;22(2):269\u2013271.10.1161\/01.STR.22.2.2692003292"},{"key":"b86","series-title":"World Neurosurg","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1016\/j.wneu.2017.08.176","article-title":"Moyamoya vasculopathy in PHACE syndrome: six new cases and review of the literature","volume":"108","author":"Tortora D","year":"2017","unstructured":"Tortora D, Severino M, Accogli A, Martinetti C, Vercellino N, Capra V, Moyamoya vasculopathy in PHACE syndrome: six new cases and review of the literature. World Neurosurg. 2017;108:291\u2013302.10.1016\/j.wneu.2017.08.17628887276"},{"issue":"1","key":"b87","series-title":"Br J Dermatol","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1111\/bjd.12435","article-title":"Pathogenesis of infantile haemangioma","volume":"169","author":"Greenberger S","year":"2013","unstructured":"Greenberger S, Bischoff J. Pathogenesis of infantile haemangioma. Br J Dermatol. 2013;169(1):12\u201319.2366847410.1111\/bjd.12435"},{"issue":"13","key":"b88","series-title":"Invest Ophthalmol Vis Sci","first-page":"1342","article-title":"Overexpression of GLUT1 and increased glucose transport leads to apoptosis in retinal endothelial cells","volume":"43","author":"Kumagai AK","year":"2002","unstructured":"Kumagai AK, Jamali JA, Deo BK, Hosoya K, Terasaki T. Overexpression of GLUT1 and increased glucose transport leads to apoptosis in retinal endothelial cells. Invest Ophthalmol Vis Sci. 2002;43(13):1342."},{"issue":"9","key":"b89","series-title":"Arterioscler Thromb Vasc Biol","doi-asserted-by":"publisher","first-page":"1646","DOI":"10.1161\/ATVBAHA.117.309510","article-title":"Endothelial cell autophagy maintains shear stress-induced nitric oxide generation via glycolysis-dependent purinergic signaling to endothelial nitric oxide synthase","volume":"37","author":"Bharath LP","year":"2017","unstructured":"Bharath LP, Cho JM, Park SK, Ruan T, Li Y, Mueller R, Endothelial cell autophagy maintains shear stress-induced nitric oxide generation via glycolysis-dependent purinergic signaling to endothelial nitric oxide synthase. Arterioscler Thromb Vasc Biol. 2017;37(9):1646\u20131656.10.1161\/ATVBAHA.117.30951028684613"},{"issue":"1","key":"b90","series-title":"Biomol Ther (Seoul)","doi-asserted-by":"publisher","first-page":"45","DOI":"10.4062\/biomolther.2019.119","article-title":"The role of a neurovascular signaling pathway involving hypoxia-inducible factor and notch in the function of the central nervous system","volume":"28","author":"Kim S","year":"2020","unstructured":"Kim S, Lee M, Choi YK. The role of a neurovascular signaling pathway involving hypoxia-inducible factor and notch in the function of the central nervous system. Biomol Ther (Seoul). 2020;28(1):45\u201357.10.4062\/biomolther.2019.119"},{"issue":"7","key":"b1","series-title":"PLoS One","doi-asserted-by":"publisher","first-page":"e22542","DOI":"10.1371\/journal.pone.0022542","article-title":"Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development","volume":"6","author":"Liu W","year":"2011","unstructured":"Liu W, Morito D, Takashima S, Mineharu Y, Kobayashi H, Hitomi T, Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development. PLoS One. 2011;6(7):e22542.2179989210.1371\/journal.pone.0022542"},{"issue":"7","key":"b2","series-title":"PLoS Med","doi-asserted-by":"publisher","first-page":"e1000097","DOI":"10.1371\/journal.pmed.1000097","article-title":"Preferred Reporting Items for Systematic Reviews and Meta-Analyses: the PRISMA statement","volume":"6","author":"Moher D","year":"2009","unstructured":"Moher D, Liberati A, Tetzlaff J, Altman DG; PRISMA Group. Preferred Reporting Items for Systematic Reviews and Meta-Analyses: the PRISMA statement. PLoS Med. 2009;6(7):e1000097.10.1371\/journal.pmed.100009719621072"},{"key":"b3","series-title":"Thieme","article-title":"Pediatric Neurovascular Disease: Surgical, Endovascular, and Medical Management","author":"Alexander MJ","year":"2011","unstructured":"Alexander MJ, Spetzler RF. Pediatric Neurovascular Disease: Surgical, Endovascular, and Medical Management. Thieme;2011."},{"issue":"2","key":"b4","series-title":"J Stroke","doi-asserted-by":"publisher","first-page":"144","DOI":"10.5853\/jos.2015.17.2.144","article-title":"A functional perspective on the embryology and anatomy of the cerebral blood supply","volume":"17","author":"Menshawi K","year":"2015","unstructured":"Menshawi K, Mohr JP, Gutierrez J. A functional perspective on the embryology and anatomy of the cerebral blood supply. J Stroke. 2015;17(2):144\u2013158.10.5853\/jos.2015.17.2.14426060802"},{"issue":"5","key":"b5","series-title":"Acta Neurochir (Wien)","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1007\/s00701-015-2367-y","article-title":"Morphology and related hemodynamics of the internal carotid arteries of moyamoya patients","volume":"157","author":"Kim T","year":"2015","unstructured":"Kim T, Bang JS, Kwon OK, Hwang G, Kim JE, Kang HS, Morphology and related hemodynamics of the internal carotid arteries of moyamoya patients. Acta Neurochir (Wien). 2015;157(5):755\u2013761.10.1007\/s00701-015-2367-y"},{"issue":"16","key":"b6","series-title":"Comput Methods Biomech Biomed Engin","doi-asserted-by":"publisher","first-page":"1760","DOI":"10.1080\/10255842.2016.1184655","article-title":"Changes to the geometry and fluid mechanics of the carotid siphon in the pediatric moyamoya disease","volume":"19","author":"Jamil M","year":"2016","unstructured":"Jamil M, Tan GXY, Huq M, Kang H, Lee ZR, Tang PH, Changes to the geometry and fluid mechanics of the carotid siphon in the pediatric moyamoya disease. Comput Methods Biomech Biomed Engin. 2016;19(16):1760\u20131771.10.1080\/10255842.2016.1184655"},{"issue":"1","key":"b7","series-title":"Sci Rep","doi-asserted-by":"publisher","first-page":"8614","DOI":"10.1038\/s41598-019-45141-y","article-title":"Vascular tortuosity of the internal carotid artery is related to the RNF213 c.14429G > A variant in moyamoya disease","volume":"9","author":"An S","year":"2019","unstructured":"An S, Kim T, Oh CW, Bang JS, Lee SU, Heo J. Vascular tortuosity of the internal carotid artery is related to the RNF213 c.14429G > A variant in moyamoya disease. Sci Rep. 2019;9(1):8614.10.1038\/s41598-019-45141-y"},{"issue":"1","key":"b8","series-title":"Sci Rep","doi-asserted-by":"publisher","first-page":"16161","DOI":"10.1038\/srep16161","article-title":"Neuromuscular regulation in zebrafish by a large AAA+ ATPase\/ubiquitin ligase, mysterin\/RNF213","volume":"5","author":"Kotani Y","year":"2015","unstructured":"Kotani Y, Morito D, Yamazaki S, Ogino K, Kawakami K, Takashima S, Neuromuscular regulation in zebrafish by a large AAA+ ATPase\/ubiquitin ligase, mysterin\/RNF213. Sci Rep. 2015;5(1):16161.10.1038\/srep1616126530008"},{"issue":"18","key":"b9","series-title":"Neuroreport","doi-asserted-by":"publisher","first-page":"1442","DOI":"10.1097\/WNR.0000000000000289","article-title":"Increased vascular MMP-9 in mice lacking RNF213: moyamoya disease susceptibility gene","volume":"25","author":"Sonobe S","year":"2014","unstructured":"Sonobe S, Fujimura M, Niizuma K, Fujimura T, Furudate S, Nishijima Y, Increased vascular MMP-9 in mice lacking RNF213: moyamoya disease susceptibility gene. Neuroreport. 2014;25(18):1442\u20131446.2538346110.1097\/WNR.0000000000000289"},{"issue":"12","key":"b10","series-title":"Arq Neuropsiquiatr","doi-asserted-by":"publisher","first-page":"917","DOI":"10.1590\/S0004-282X2012001200003","article-title":"Carotid siphon geometry and variants of the circle of Willis in the origin of carotid aneurysms","volume":"70","author":"Silva Neto \u00c2R","year":"2012","unstructured":"Silva Neto \u00c2R, C\u00e2mara RLB, Valen\u00e7a MM. Carotid siphon geometry and variants of the circle of Willis in the origin of carotid aneurysms. Arq Neuropsiquiatr. 2012;70(12):917\u2013921.10.1590\/S0004-282X2012001200003"},{"issue":"9","key":"b11","series-title":"AJNR Am J Neuroradiol","doi-asserted-by":"publisher","first-page":"1771","DOI":"10.3174\/ajnr.A5285","article-title":"Influence of carotid siphon anatomy on brain aneurysm presentation","volume":"38","author":"Waihrich E","year":"2017","unstructured":"Waihrich E, Clavel P, Mendes GAC, Iosif C, Moraes Kessler I, Mounayer C. Influence of carotid siphon anatomy on brain aneurysm presentation. AJNR Am J Neuroradiol. 2017;38(9):1771\u20131775.10.3174\/ajnr.A528528684458"},{"key":"b12","first-page":"72","article-title":"Moyamoya disease: using computational fluid dynamics to propose a novel \u201cmechanical theory\u201d of pathogenesis","author":"Sudhir BJ","year":"2018","unstructured":"Sudhir BJ, Kumar KS. Moyamoya disease: using computational fluid dynamics to propose a novel \u201cmechanical theory\u201d of pathogenesis. In: 2018 International Conference on Intelligent Informatics and Biomedical Sciences (ICIIBMS).IEEE;2018:72\u201377."},{"issue":"12","key":"b13","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"2263","DOI":"10.1007\/s00381-013-2123-7","article-title":"Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery","volume":"29","author":"Lee JY","year":"2013","unstructured":"Lee JY, Kim SK, Cheon JE, Choi JW, Phi JH, Kim IO, Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery. Childs Nerv Syst. 2013;29(12):2263\u20132269.10.1007\/s00381-013-2123-723653141"},{"issue":"8","key":"b14","series-title":"PLoS One","doi-asserted-by":"publisher","first-page":"e0202526","DOI":"10.1371\/journal.pone.0202526","article-title":"Increased MMP activity in curved geometries disrupts the endothelial cell glycocalyx creating a proinflammatory environment","volume":"13","author":"Cooper S","year":"2018","unstructured":"Cooper S, Emmott A, McDonald KK, Campeau MA, Leask RL. Increased MMP activity in curved geometries disrupts the endothelial cell glycocalyx creating a proinflammatory environment. PLoS One. 2018;13(8):e0202526.3013840010.1371\/journal.pone.0202526"},{"issue":"3","key":"b15","series-title":"J Vasc Surg","doi-asserted-by":"publisher","first-page":"601","DOI":"10.1016\/j.jvs.2003.10.058","article-title":"Arterial enlargement, tortuosity, and intimal thickening in response to sequential exposure to high and low wall shear stress","volume":"39","author":"Sho E","year":"2004","unstructured":"Sho E, Nanjo H, Sho M, Kobayashi M, Komatsu M, Kawamura K, Arterial enlargement, tortuosity, and intimal thickening in response to sequential exposure to high and low wall shear stress. J Vasc Surg. 2004;39(3):601\u2013612.1498145510.1016\/j.jvs.2003.10.058"},{"issue":"2","key":"b16","series-title":"Exp Mol Pathol","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1006\/exmp.2002.2426","article-title":"Subnormal shear stress-induced intimal thickening requires medial smooth muscle cell proliferation and migration","volume":"72","author":"Sho M","year":"2002","unstructured":"Sho M, Sho E, Singh TM, Komatsu M, Sugita A, Xu C, Subnormal shear stress-induced intimal thickening requires medial smooth muscle cell proliferation and migration. Exp Mol Pathol. 2002;72(2):150\u2013160.10.1006\/exmp.2002.242611890724"},{"issue":"3","key":"b17","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"689","DOI":"10.3171\/2015.6.JNS15933","article-title":"Sonographic findings associated with stenosis progression and vascular complications in moyamoya disease","volume":"125","author":"Lee WJ","year":"2016","unstructured":"Lee WJ, Jung KH, Lee KJ, Kim JM, Lee ST, Chu K, Sonographic findings associated with stenosis progression and vascular complications in moyamoya disease. J Neurosurg. 2016;125(3):689\u2013697.10.3171\/2015.6.JNS1593326654185"},{"issue":"1","key":"b18","series-title":"Hellenic J Cardiol","first-page":"9","article-title":"Vascular wall shear stress: basic principles and methods","volume":"46","author":"Papaioannou TG","year":"2005","unstructured":"Papaioannou TG, Stefanadis C. Vascular wall shear stress: basic principles and methods. Hellenic J Cardiol. 2005;46(1):9\u201315.15807389"},{"issue":"8","key":"b19","series-title":"PLoS One","doi-asserted-by":"publisher","first-page":"e0161611","DOI":"10.1371\/journal.pone.0161611","article-title":"Time-series interactions of gene expression, vascular growth and hemodynamics during early embryonic arterial development","volume":"11","author":"Goktas S","year":"2016","unstructured":"Goktas S, Uslu FE, Kowalski WJ, Ermek E, Keller BB, Pekkan K. Time-series interactions of gene expression, vascular growth and hemodynamics during early embryonic arterial development. PLoS One. 2016;11(8):e0161611.2755215010.1371\/journal.pone.0161611"},{"issue":"1","key":"b20","series-title":"J Cell Physiol","doi-asserted-by":"publisher","first-page":"229","DOI":"10.1002\/jcp.20658","article-title":"Nitric oxide and p38 MAP kinase mediate shear stress-dependent inhibition of MMP-2 production in microvascular endothelial cells","volume":"208","author":"Milkiewicz M","year":"2006","unstructured":"Milkiewicz M, Kelland C, Colgan S, Haas TL. Nitric oxide and p38 MAP kinase mediate shear stress-dependent inhibition of MMP-2 production in microvascular endothelial cells. J Cell Physiol. 2006;208(1):229\u2013237.10.1002\/jcp.2065816575906"},{"issue":"8","key":"b21","series-title":"PLoS Comput Biol","doi-asserted-by":"publisher","first-page":"e1008161","DOI":"10.1371\/journal.pcbi.1008161","article-title":"Cell signaling model for arterial mechanobiology","volume":"16","author":"Irons L","year":"2020","unstructured":"Irons L, Humphrey JD. Cell signaling model for arterial mechanobiology. PLoS Comput Biol. 2020;16(8):e1008161.10.1371\/journal.pcbi.100816132834001"},{"issue":"5","key":"b22","series-title":"Surg Neurol","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1016\/j.surneu.2008.10.009","article-title":"Increased expression of serum matrix metalloproteinase-9 in patients with moyamoya disease","volume":"72","author":"Fujimura M","year":"2009","unstructured":"Fujimura M, Watanabe M, Narisawa A, Shimizu H, Tominaga T. Increased expression of serum matrix metalloproteinase-9 in patients with moyamoya disease. Surg Neurol. 2009;72(5):476\u2013480.1914719610.1016\/j.surneu.2008.10.009"},{"issue":"6","key":"b23","series-title":"J Neurol Neurosurg Psychiatry","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1136\/jnnp.2009.191817","article-title":"Plasma matrix metalloproteinases, cytokines and angiogenic factors in moyamoya disease","volume":"81","author":"Kang HS","year":"2010","unstructured":"Kang HS, Kim JH, Phi JH, Kim YY, Kim JE, Wang KC, Plasma matrix metalloproteinases, cytokines and angiogenic factors in moyamoya disease. J Neurol Neurosurg Psychiatry. 2010;81(6):673\u2013678.1996584410.1136\/jnnp.2009.191817"},{"issue":"6","key":"b24","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"1074","DOI":"10.1227\/01.NEU.0000215854.66011.4F","article-title":"Single nucleotide polymorphisms of tissue inhibitor of metalloproteinase genes in familial moyamoya disease","volume":"58","author":"Kang HS","year":"2006","unstructured":"Kang HS, Kim SK, Cho BK, Kim YY, Hwang YS, Wang KC. Single nucleotide polymorphisms of tissue inhibitor of metalloproteinase genes in familial moyamoya disease. Neurosurgery. 2006;58(6):1074\u20131080.10.1227\/01.NEU.0000215854.66011.4F16723886"},{"key":"b25","series-title":"BMC Neurol","doi-asserted-by":"publisher","first-page":"180","DOI":"10.1186\/s12883-014-0180-5","article-title":"The GC + CC genotype at position -418 in TIMP-2 promoter and the -1575GA\/-1306CC genotype in MMP-2 is genetic predisposing factors for prevalence of moyamoya disease","volume":"14","author":"Park YS","year":"2014","unstructured":"Park YS, Jeon YJ, Kim HS, Han IB, Oh SH, Kim DS, Kim NK. The GC + CC genotype at position -418 in TIMP-2 promoter and the -1575GA\/-1306CC genotype in MMP-2 is genetic predisposing factors for prevalence of moyamoya disease. BMC Neurol. 2014;14:180.10.1186\/s12883-014-0180-525280484"},{"key":"b26","series-title":"J Neurol Sci","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1016\/j.jns.2017.08.3380","article-title":"Concurrent moyamoya syndrome and Graves\u2019 disease: a case report","volume":"381","author":"Fu MH","year":"2017","unstructured":"Fu MH. Concurrent moyamoya syndrome and Graves\u2019 disease: a case report. J Neurol Sci. 2017;381:413.10.1016\/j.jns.2017.08.3380"},{"issue":"6","key":"b27","series-title":"J Stroke Cerebrovasc Dis","doi-asserted-by":"publisher","first-page":"528","DOI":"10.1016\/j.jstrokecerebrovasdis.2010.03.006","article-title":"Moyamoya syndrome associated with Graves\u2019 disease: a case report and review of the literature","volume":"20","author":"Malik S","year":"2011","unstructured":"Malik S, Russman AN, Katramados AM, Silver B, Mitsias PD. Moyamoya syndrome associated with Graves\u2019 disease: a case report and review of the literature. J Stroke Cerebrovasc Dis. 2011;20(6):528\u2013536.2113066810.1016\/j.jstrokecerebrovasdis.2010.03.006"},{"issue":"1","key":"b28","series-title":"Adv Clin Exp Med","doi-asserted-by":"publisher","first-page":"99","DOI":"10.17219\/acem\/68991","article-title":"Elevated serum concentrations of metalloproteinases (MMP-2, MMP-9) and their inhibitors (TIMP-1, TIMP-2) in patients with Graves\u2019 orbitopathy","volume":"27","author":"Kapelko-S\u0142owik K","year":"2018","unstructured":"Kapelko-S\u0142owik K, S\u0142owik M, Szali\u0144ski M, Dybko J, Wo\u0142owiec D, Prajs I, Elevated serum concentrations of metalloproteinases (MMP-2, MMP-9) and their inhibitors (TIMP-1, TIMP-2) in patients with Graves\u2019 orbitopathy. Adv Clin Exp Med. 2018;27(1):99\u2013103.10.17219\/acem\/6899129521049"},{"issue":"4","key":"b29","series-title":"Int J Radiat Oncol Biol Phys","doi-asserted-by":"publisher","first-page":"1222","DOI":"10.1016\/j.ijrobp.2006.01.038","article-title":"Radiation-induced moyamoya syndrome","volume":"65","author":"Desai SS","year":"2006","unstructured":"Desai SS, Paulino AC, Mai WY, Teh BS. Radiation-induced moyamoya syndrome. Int J Radiat Oncol Biol Phys. 2006;65(4):1222\u20131227.1662689010.1016\/j.ijrobp.2006.01.038"},{"issue":"6","key":"b30","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1159\/000446075","article-title":"Radiation-induced moyamoya syndrome after proton beam therapy in the pediatric patient: a case series","volume":"51","author":"Reynolds MR","year":"2016","unstructured":"Reynolds MR, Haydon DH, Caird J, Leonard JR. Radiation-induced moyamoya syndrome after proton beam therapy in the pediatric patient: a case series. Pediatr Neurosurg. 2016;51(6):297\u2013301.10.1159\/00044607527193755"},{"issue":"12","key":"b31","series-title":"Eur J Case Rep Intern Med","first-page":"001337","article-title":"Moyamoya syndrome after radiation therapy: a clinical report","volume":"6","author":"Almeida P","year":"2019","unstructured":"Almeida P, Rocha A, Alves G, Parreira T, Silva ML, Cerejo A, Moyamoya syndrome after radiation therapy: a clinical report. Eur J Case Rep Intern Med. 2019;6(12):001337.31893204"},{"issue":"5","key":"b32","series-title":"Int J Cancer","doi-asserted-by":"publisher","first-page":"766","DOI":"10.1002\/1097-0215(20001201)88:5<766::AID-IJC13>3.0.CO;2-Y","article-title":"Effects of radiation on the levels of MMP-2, MMP-9 and TIMP-1 during morphogenic glial-endothelial cell interactions","volume":"88","author":"Nirmala C","year":"2000","unstructured":"Nirmala C, Jasti SL, Sawaya R, Kyritsis AP, Konduri SD, Ali-Osman F, Effects of radiation on the levels of MMP-2, MMP-9 and TIMP-1 during morphogenic glial-endothelial cell interactions. Int J Cancer. 2000;88(5):766\u2013771.1107224610.1002\/1097-0215(20001201)88:5<766::AID-IJC13>3.0.CO;2-Y"},{"issue":"2","key":"b33","series-title":"Radiat Res","doi-asserted-by":"publisher","first-page":"174","DOI":"10.1667\/RR3107","article-title":"Retinoids and TIMP1 prevent radiation-induced apoptosis of capillary endothelial cells","volume":"161","author":"Vorotnikova E","year":"2004","unstructured":"Vorotnikova E, Tries M, Braunhut S. Retinoids and TIMP1 prevent radiation-induced apoptosis of capillary endothelial cells. Radiat Res. 2004;161(2):174\u2013184.1473107210.1667\/RR3107"},{"issue":"19","key":"b34","series-title":"Curr Med Chem","doi-asserted-by":"publisher","first-page":"2059","DOI":"10.2174\/092986710791233706","article-title":"Regulation of the protein C anticoagulant and antiinflammatory pathways","volume":"17","author":"Rezaie AR","year":"2010","unstructured":"Rezaie AR. Regulation of the protein C anticoagulant and antiinflammatory pathways. Curr Med Chem. 2010;17(19):2059\u20132069.10.2174\/09298671079123370620423310"},{"issue":"8","key":"b35","series-title":"Am J Physiol Cell Physiol","doi-asserted-by":"publisher","first-page":"C1109","DOI":"10.1152\/ajpcell.00369.2011","article-title":"Endothelial cells express a unique transcriptional profile under very high wall shear stress known to induce expansive arterial remodeling","volume":"302","author":"Dolan JM","year":"2012","unstructured":"Dolan JM, Sim FJ, Meng H, Kolega J. Endothelial cells express a unique transcriptional profile under very high wall shear stress known to induce expansive arterial remodeling. Am J Physiol Cell Physiol. 2012;302(8):C1109\u2013C1118.10.1152\/ajpcell.00369.201122173868"},{"issue":"suppl 1","key":"b36","series-title":"Saudi Med J","first-page":"S69","article-title":"Moyamoya syndrome as a risk factor for stroke in Saudi children Novel and usual associations","volume":"27","author":"Salih MA","year":"2006","unstructured":"Salih MA, Murshid WR, Al-Salman MM, Abdel-Gader AG, Al-Jarallah AA, Alorainy IA, Moyamoya syndrome as a risk factor for stroke in Saudi children Novel and usual associations. Saudi Med J. 2006;27(suppl 1):S69\u2013S80.16532134"},{"issue":"9","key":"b37","series-title":"Am J Hematol","doi-asserted-by":"publisher","first-page":"618","DOI":"10.1002\/ajh.21475","article-title":"Vasculopathy in sickle cell disease: biology, pathophysiology, genetics, translational medicine, and new research directions","volume":"84","author":"Kato GJ","year":"2009","unstructured":"Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: biology, pathophysiology, genetics, translational medicine, and new research directions. Am J Hematol. 2009;84(9):618\u2013625.10.1002\/ajh.2147519610078"},{"issue":"5","key":"b38","series-title":"Med Biol Eng Comput","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1007\/s11517-008-0330-2","article-title":"Wall shear stress as measured in vivo: consequences for the design of the arterial system","volume":"46","author":"Reneman RS","year":"2008","unstructured":"Reneman RS, Hoeks APG. Wall shear stress as measured in vivo: consequences for the design of the arterial system. Med Biol Eng Comput. 2008;46(5):499\u2013507.10.1007\/s11517-008-0330-218324431"},{"key":"b39","series-title":"Annu Rev Pathol","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1146\/annurev-pathmechdis-012418-012838","article-title":"Pathophysiology of sickle cell disease","volume":"14","author":"Sundd P","year":"2019","unstructured":"Sundd P, Gladwin MT, Novelli EM. Pathophysiology of sickle cell disease. Annu Rev Pathol. 2019;14:263\u2013292.3033256210.1146\/annurev-pathmechdis-012418-012838"},{"issue":"6","key":"b40","series-title":"Blood","doi-asserted-by":"publisher","first-page":"1584","DOI":"10.1182\/blood.V97.6.1584","article-title":"Endothelial dysfunction in patients with sickle cell disease is related to selective impairment of shear stress-mediated vasodilation","volume":"97","author":"Belhassen L","year":"2001","unstructured":"Belhassen L, Pelle G, Sediame S, Bachir D, Carville C, Bucherer C, Endothelial dysfunction in patients with sickle cell disease is related to selective impairment of shear stress-mediated vasodilation. Blood. 2001;97(6):1584\u20131589.10.1182\/blood.V97.6.158411238095"},{"issue":"11","key":"b41","series-title":"Blood","doi-asserted-by":"publisher","first-page":"1220","DOI":"10.1182\/blood.V108.11.1220.1220","article-title":"Increased levels and activities of matrix metalloproteinases in sickle cell disease","volume":"108","author":"Franco-Penteado CF","year":"2006","unstructured":"Franco-Penteado CF, Hyslop S, Conran N, Saad STO, Costa FF. Increased levels and activities of matrix metalloproteinases in sickle cell disease. Blood. 2006;108(11):1220.10.1182\/blood.V108.11.1220.1220"},{"issue":"12","key":"b42","series-title":"Circulation","doi-asserted-by":"publisher","first-page":"1530","DOI":"10.1161\/01.CIR.0000121741.50315.C2","article-title":"Increased carotid wall stress in vascular Ehlers-Danlos syndrome","volume":"109","author":"Boutouyrie P","year":"2004","unstructured":"Boutouyrie P, Germain DP, Fiessinger JN, Laloux B, Perdu J, Laurent S. Increased carotid wall stress in vascular Ehlers-Danlos syndrome. Circulation. 2004;109(12):1530\u20131535.10.1161\/01.CIR.0000121741.50315.C215007000"},{"issue":"6","key":"b43","series-title":"J Vasc Surg","doi-asserted-by":"publisher","first-page":"1263","DOI":"10.1067\/mva.2001.113579","article-title":"The role of type I collagen in aortic wall strength with a homotrimeric [\u03b11(I)]3 collagen mouse model","volume":"33","author":"Vouyouka AG","year":"2001","unstructured":"Vouyouka AG, Pfeiffer BJ, Liem TK, Taylor TA, Mudaliar J, Phillips CL. The role of type I collagen in aortic wall strength with a homotrimeric [\u03b11(I)]3 collagen mouse model. J Vasc Surg. 2001;33(6):1263\u20131270.10.1067\/mva.2001.11357911389427"},{"issue":"4","key":"b44","series-title":"AJNR Am J Neuroradiol","first-page":"748","article-title":"Abnormalities in the cerebral arterial system in osteogenesis imperfecta","volume":"24","author":"Albayram S","year":"2003","unstructured":"Albayram S, Kizilkilic O, Yilmaz H, Tuysuz B, Kocer N, Islak C. Abnormalities in the cerebral arterial system in osteogenesis imperfecta. AJNR Am J Neuroradiol. 2003;24(4):748\u2013750.12695216"},{"issue":"7","key":"b45","series-title":"Radiographics","doi-asserted-by":"publisher","first-page":"2101","DOI":"10.1148\/rg.327125716","article-title":"Craniospinal abnormalities and neurologic complications of osteogenesis imperfecta: imaging overview","volume":"32","author":"Khandanpour N","year":"2012","unstructured":"Khandanpour N, Connolly DJA, Raghavan A, Griffiths PD, Hoggard N. Craniospinal abnormalities and neurologic complications of osteogenesis imperfecta: imaging overview. Radiographics. 2012;32(7):2101\u20132112.2315086010.1148\/rg.327125716"},{"issue":"5","key":"b46","series-title":"Circ Res","doi-asserted-by":"publisher","first-page":"512","DOI":"10.1161\/CIRCRESAHA.107.157776","article-title":"Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in the thoracic aortic aneurysm in Marfan syndrome","volume":"101","author":"Chung AWY","year":"2007","unstructured":"Chung AWY, Au Yeung K, Sandor GGS, Judge DP, Dietz HC, van Breemen C. Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in the thoracic aortic aneurysm in Marfan syndrome. Circ Res. 2007;101(5):512\u2013522.10.1161\/CIRCRESAHA.107.15777617641224"},{"issue":"2","key":"b47","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"210","DOI":"10.1227\/NEU.0b013e31827b9beb","article-title":"Prevalence and characteristics of concurrent down syndrome in patients with moyamoya disease","volume":"72","author":"Kainth DS","year":"2013","unstructured":"Kainth DS, Chaudhry SA, Kainth HS, Suri FK, Qureshi AI. Prevalence and characteristics of concurrent down syndrome in patients with moyamoya disease. Neurosurgery. 2013;72(2):210\u2013215.2314996610.1227\/NEU.0b013e31827b9beb"},{"issue":"5","key":"b48","series-title":"Pediatrics","doi-asserted-by":"publisher","first-page":"e694","DOI":"10.1542\/peds.2005-0568","article-title":"Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization","volume":"116","author":"Jea A","year":"2005","unstructured":"Jea A, Smith ER, Robertson R, Scott RM. Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization. Pediatrics. 2005;116(5):e694\u2013e701.10.1542\/peds.2005-056816263984"},{"issue":"10","key":"b49","series-title":"FEBS J","doi-asserted-by":"publisher","first-page":"2418","DOI":"10.1111\/febs.12220","article-title":"New insights into the pathobiology of Down syndrome\u2014hyaluronan synthase-2 overexpression is regulated by collagen VI \u03b12 chain","volume":"280","author":"Karousou E","year":"2013","unstructured":"Karousou E, Stachtea X, Moretto P, Viola M, Vigetti D, D\u2019Angelo ML, New insights into the pathobiology of Down syndrome\u2014hyaluronan synthase-2 overexpression is regulated by collagen VI \u03b12 chain. FEBS J. 2013;280(10):2418\u20132430.10.1111\/febs.1222023452080"},{"issue":"34","key":"b50","series-title":"Proc Natl Acad Sci U S A","doi-asserted-by":"publisher","first-page":"20753","DOI":"10.1073\/pnas.2004281117","article-title":"Identifying collagen VI as a target of fibrotic diseases regulated by CREBBP\/EP300","volume":"117","author":"Williams LM","year":"2020","unstructured":"Williams LM, McCann FE, Cabrita MA, Layton T, Cribbs A, Knezevic B, Identifying collagen VI as a target of fibrotic diseases regulated by CREBBP\/EP300. Proc Natl Acad Sci U S A. 2020;117(34):20753\u201320763.10.1073\/pnas.2004281117"},{"issue":"11","key":"b51","series-title":"Biochim Biophys Acta","doi-asserted-by":"publisher","first-page":"2106","DOI":"10.1016\/j.bbadis.2014.07.008","article-title":"Vascular wall extracellular matrix proteins and vascular diseases","volume":"1842","author":"Xu J","year":"2014","unstructured":"Xu J, Shi GP. Vascular wall extracellular matrix proteins and vascular diseases. Biochim Biophys Acta. 2014;1842(11):2106\u20132119.2504585410.1016\/j.bbadis.2014.07.008"},{"key":"b52","series-title":"Vasc Health Risk Manag","doi-asserted-by":"publisher","first-page":"375","DOI":"10.2147\/VHRM.S21273","article-title":"Stiffness of the large arteries in individuals with and without Down syndrome","volume":"7","author":"Rodrigues AN","year":"2011","unstructured":"Rodrigues AN, Coelho LC, Goncalves WL, Gouvea SA, Vasconcellos MJ, Cunha RS, Abreu GR. Stiffness of the large arteries in individuals with and without Down syndrome. Vasc Health Risk Manag. 2011;7:375\u2013381.10.2147\/VHRM.S2127321731889"},{"issue":"2","key":"b53","series-title":"J Ment Defic Res","first-page":"123","article-title":"Blood pressure in Down\u2019s syndrome","volume":"23","author":"Richards BW","year":"1979","unstructured":"Richards BW, Enver F. Blood pressure in Down\u2019s syndrome. J Ment Defic Res. 1979;23(2):123\u2013135.158658"},{"issue":"4","key":"b54","series-title":"Pediatrics","doi-asserted-by":"publisher","first-page":"e20180840","DOI":"10.1542\/peds.2018-0840","article-title":"Blood pressure elevation and risk of moyamoya syndrome in patients with trisomy 21","volume":"142","author":"Santoro JD","year":"2018","unstructured":"Santoro JD, Lee S, Mlynash M, Nguyen T, Lazzareschi DV, Kraler LD, Blood pressure elevation and risk of moyamoya syndrome in patients with trisomy 21. Pediatrics. 2018;142(4):e20180840.3019034710.1542\/peds.2018-0840"},{"issue":"1","key":"b55","series-title":"Neurobiol Dis","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1006\/nbdi.1995.0002","article-title":"Expression of the neurofibromatosis I gene product, neurofibromin, in blood vessel endothelial cells and smooth muscle","volume":"2","author":"Norton KK","year":"1995","unstructured":"Norton KK, Xu J, Gutmann DH. Expression of the neurofibromatosis I gene product, neurofibromin, in blood vessel endothelial cells and smooth muscle. Neurobiol Dis. 1995;2(1):13\u201321.10.1006\/nbdi.1995.00028980005"},{"issue":"3","key":"b56","series-title":"Genet Med","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1097\/00125817-200205000-00002","article-title":"Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force","volume":"4","author":"Friedman JM","year":"2002","unstructured":"Friedman JM, Arbiser J, Epstein JA, Gutmann DH, Huot SJ, Lin AE, Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force. Genet Med. 2002;4(3):105\u2013111.1218014310.1097\/00125817-200205000-00002"},{"key":"b57","article-title":"MedlinePlus Genetics","author":"HRAS gene","unstructured":"HRAS gene. MedlinePlus Genetics. Accessed July 12, 2021.https:\/\/medlineplus.gov\/genetics\/gene\/hras\/"},{"issue":"4","key":"b58","series-title":"Arterioscler Thromb Vasc Biol","doi-asserted-by":"publisher","first-page":"582","DOI":"10.1161\/01.ATV.0000064372.78561.A5","article-title":"Elastogenesis in human arterial disease: a role for macrophages in disordered elastin synthesis","volume":"23","author":"Krettek A","year":"2003","unstructured":"Krettek A, Sukhova GK, Libby P. Elastogenesis in human arterial disease: a role for macrophages in disordered elastin synthesis. Arterioscler Thromb Vasc Biol. 2003;23(4):582\u2013587.1261567410.1161\/01.ATV.0000064372.78561.A5"},{"issue":"5","key":"b59","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1016\/j.pediatrneurol.2004.12.010","article-title":"Costello syndrome showing moyamoya-like vasculopathy","volume":"32","author":"Shiihara T","year":"2005","unstructured":"Shiihara T, Kato M, Mitsuhashi Y, Hayasaka K. Costello syndrome showing moyamoya-like vasculopathy. Pediatr Neurol. 2005;32(5):361\u2013363.10.1016\/j.pediatrneurol.2004.12.01015866441"},{"issue":"4","key":"b60","series-title":"Dermatology","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1159\/000051558","article-title":"Costello syndrome with decreased gene expression of elastin in cultured dermal fibroblasts","volume":"201","author":"Hatamochi A","year":"2000","unstructured":"Hatamochi A, Nagayama H, Kuroda K, Shinkai H, Ishikiriyama S, Kobayashi M, Kobayashi K. Costello syndrome with decreased gene expression of elastin in cultured dermal fibroblasts. Dermatology. 2000;201(4):366\u2013369.10.1159\/00005155811146354"},{"issue":"3","key":"b61","series-title":"Curr Opin Genet Dev","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1016\/j.gde.2009.04.001","article-title":"The RASopathies: developmental syndromes of Ras\/MAPK pathway dysregulation","volume":"19","author":"Tidyman WE","year":"2009","unstructured":"Tidyman WE, Rauen KA. The RASopathies: developmental syndromes of Ras\/MAPK pathway dysregulation. Curr Opin Genet Dev. 2009;19(3):230\u2013236.1946785510.1016\/j.gde.2009.04.001"},{"issue":"2","key":"b62","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1016\/j.pediatrneurol.2011.03.007","article-title":"Moyamoya syndrome in a child with Noonan syndrome","volume":"45","author":"Hung PC","year":"2011","unstructured":"Hung PC, Wang HS, Wong AMC. Moyamoya syndrome in a child with Noonan syndrome. Pediatr Neurol. 2011;45(2):129\u2013131.10.1016\/j.pediatrneurol.2011.03.007"},{"issue":"4","key":"b63","series-title":"Brain Dev","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/S0387-7604(02)00014-1","article-title":"Cardio-facio-cutaneous syndrome and moyamoya syndrome","volume":"24","author":"Ishiguro Y","year":"2002","unstructured":"Ishiguro Y, Kubota T, Takenaka J, Maruyama K, Okumura A, Negoro T, Watanabe K. Cardio-facio-cutaneous syndrome and moyamoya syndrome. Brain Dev. 2002;24(4):245\u2013249.10.1016\/S0387-7604(02)00014-112015168"},{"issue":"1","key":"b64","series-title":"Am J Med Genet A","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1002\/ajmg.a.61921","article-title":"Moyamoya syndrome in a child with Legius syndrome: Introducing a cerebral vasculopathy to the SPRED1 phenotype?","volume":"185","author":"Pabst L","year":"2021","unstructured":"Pabst L, Carroll J, Lo W, Truxal KV. Moyamoya syndrome in a child with Legius syndrome: Introducing a cerebral vasculopathy to the SPRED1 phenotype?. Am J Med Genet A. 2021;185(1):223\u2013227.10.1002\/ajmg.a.61921"},{"issue":"2","key":"b65","series-title":"Pediatrics","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1542\/peds.103.2.505","article-title":"Moyamoya syndrome in children with Alagille syndrome: additional evidence of a vasculopathy","volume":"103","author":"Woolfenden AR","year":"1999","unstructured":"Woolfenden AR, Albers GW, Steinberg GK, Hahn JS, Johnston DC, Farrell K. Moyamoya syndrome in children with Alagille syndrome: additional evidence of a vasculopathy. Pediatrics. 1999;103(2):505\u2013508.992585310.1542\/peds.103.2.505"},{"issue":"1","key":"b66","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1159\/000204907","article-title":"Identification of an association between Robinow syndrome and moyamoya","volume":"45","author":"Qaiser R","year":"2009","unstructured":"Qaiser R, Scott RM, Smith ER. Identification of an association between Robinow syndrome and moyamoya. Pediatr Neurosurg. 2009;45(1):69\u201372.1925873310.1159\/000204907"},{"key":"b67","series-title":"Int J Vasc Med","first-page":"293641","article-title":"Signaling required for blood vessel maintenance: molecular basis and pathological manifestations","volume":"2012","author":"Murakami M","year":"2012","unstructured":"Murakami M. Signaling required for blood vessel maintenance: molecular basis and pathological manifestations. Int J Vasc Med. 2012;2012:293641.22187650"},{"issue":"1","key":"b68","series-title":"Dev Cell","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/j.devcel.2008.12.009","article-title":"Nrarp coordinates endothelial Notch and Wnt signaling to control vessel density in angiogenesis","volume":"16","author":"Phng LK","year":"2009","unstructured":"Phng LK, Potente M, Leslie JD, Babbage J, Nyqvist D, Lobov I, Nrarp coordinates endothelial Notch and Wnt signaling to control vessel density in angiogenesis. Dev Cell. 2009;16(1):70\u201382.1915471910.1016\/j.devcel.2008.12.009"},{"issue":"2","key":"b69","series-title":"J Med Genet","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1136\/jmg.2006.044313","article-title":"Schimke immuno-osseous dysplasia: a clinicopathological correlation","volume":"44","author":"Clewing JM","year":"2007","unstructured":"Clewing JM, Antalfy BC, L\u00fccke T, Najafian B, Marwedel KM, Hori A, Schimke immuno-osseous dysplasia: a clinicopathological correlation. J Med Genet. 2007;44(2):122\u2013130.1684056810.1136\/jmg.2006.044313"},{"issue":"10","key":"b70","series-title":"Eur J Pediatr","doi-asserted-by":"publisher","first-page":"1567","DOI":"10.1007\/s00431-012-1732-6","article-title":"Medical management of moyamoya disease and recurrent stroke in an infant with Majewski osteodysplastic primordial dwarfism type II (MOPD II)","volume":"171","author":"K\u0131l\u0131\u00e7 E","year":"2012","unstructured":"K\u0131l\u0131\u00e7 E, Utine E, \u00dcnal S, Halilo\u011flu G, O\u011fuz KK, Cetin M, Medical management of moyamoya disease and recurrent stroke in an infant with Majewski osteodysplastic primordial dwarfism type II (MOPD II). Eur J Pediatr. 2012;171(10):1567\u20131571.10.1007\/s00431-012-1732-6"},{"issue":"4","key":"b71","series-title":"J Neurosurg Pediatr","doi-asserted-by":"publisher","first-page":"320","DOI":"10.3171\/2008.12.PEDS08205","article-title":"Seckel syndrome and moyamoya","volume":"3","author":"Codd PJ","year":"2009","unstructured":"Codd PJ, Scott RM, Smith ER. Seckel syndrome and moyamoya. J Neurosurg Pediatr. 2009;3(4):320\u2013324.1933841210.3171\/2008.12.PEDS08205"},{"key":"b72","series-title":"Appl Clin Genet","first-page":"49","article-title":"Moyamoya disease and syndromes: from genetics to clinical management","volume":"8","author":"Guey S","year":"2015","unstructured":"Guey S, Tournier-Lasserve E, Herv\u00e9 D, Kossorotoff M. Moyamoya disease and syndromes: from genetics to clinical management. Appl Clin Genet. 2015;8:49\u201368.25733922"},{"issue":"6","key":"b73","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"718","DOI":"10.1016\/j.ajhg.2011.04.017","article-title":"Loss of BRCC3 deubiquitinating enzyme leads to abnormal angiogenesis and is associated with syndromic moyamoya","volume":"88","author":"Miskinyte S","year":"2011","unstructured":"Miskinyte S, Butler MG, Herv\u00e9 D, Sarret C, Nicolino M, Petralia JD, Loss of BRCC3 deubiquitinating enzyme leads to abnormal angiogenesis and is associated with syndromic moyamoya. Am J Hum Genet. 2011;88(6):718\u2013728.10.1016\/j.ajhg.2011.04.01721596366"},{"issue":"1","key":"b74","series-title":"Int J Mol Med","first-page":"29","article-title":"Expression of BRCC3, a novel cell cycle regulated molecule, is associated with increased phospho-ERK and cell proliferation","volume":"19","author":"Boudreau HE","year":"2007","unstructured":"Boudreau HE, Broustas CG, Gokhale PC, Kumar D, Mewani RR, Rone JD, Expression of BRCC3, a novel cell cycle regulated molecule, is associated with increased phospho-ERK and cell proliferation. Int J Mol Med. 2007;19(1):29\u201339.17143545"},{"issue":"10","key":"b75","series-title":"Neurol Med Chir (Tokyo)","doi-asserted-by":"publisher","first-page":"361","DOI":"10.2176\/nmc.ra.2019-0059","article-title":"Influence of inflammatory disease on the pathophysiology of moyamoya disease and quasi-moyamoya disease","volume":"59","author":"Mikami T","year":"2019","unstructured":"Mikami T, Suzuki H, Komatsu K, Mikuni N. Influence of inflammatory disease on the pathophysiology of moyamoya disease and quasi-moyamoya disease. Neurol Med Chir (Tokyo). 2019;59(10):361\u2013370.10.2176\/nmc.ra.2019-0059"},{"issue":"9","key":"b76","series-title":"J Infect Dis","doi-asserted-by":"publisher","first-page":"1411","DOI":"10.1086\/374644","article-title":"Matrix metalloproteinase-9 in pneumococcal meningitis: activation via an oxidative pathway","volume":"187","author":"Meli DN","year":"2003","unstructured":"Meli DN, Christen S, Leib SL. Matrix metalloproteinase-9 in pneumococcal meningitis: activation via an oxidative pathway. J Infect Dis. 2003;187(9):1411\u20131415.10.1086\/37464412717622"},{"issue":"1","key":"b77","series-title":"BMC Infect Dis","doi-asserted-by":"publisher","first-page":"722","DOI":"10.1186\/s12879-016-1953-9","article-title":"Title: role of matrix metalloproteinase -9 in progression of tuberculous meningitis: a pilot study in patients at different stages of the disease","volume":"16","author":"Majeed S","year":"2016","unstructured":"Majeed S, Singh P, Sharma N, Sharma S. Title: role of matrix metalloproteinase -9 in progression of tuberculous meningitis: a pilot study in patients at different stages of the disease. BMC Infect Dis. 2016;16(1):722.10.1186\/s12879-016-1953-927899068"},{"key":"b78","series-title":"Clin Neurol Neurosurg","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1016\/j.clineuro.2014.12.001","article-title":"Moyamoya in a patient with Sneddon\u2019s syndrome","volume":"129","author":"Fierini F","year":"2015","unstructured":"Fierini F, Barilaro A, Giambene B, Carlucci G, Grandi V, Maio V, Pantoni L. Moyamoya in a patient with Sneddon\u2019s syndrome. Clin Neurol Neurosurg. 2015;129:34\u201336.10.1016\/j.clineuro.2014.12.001"},{"issue":"10","key":"b79","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1056\/NEJMoa1307361","article-title":"Early-onset stroke and vasculopathy associated with mutations in ADA2","volume":"370","author":"Zhou Q","year":"2014","unstructured":"Zhou Q, Yang D, Ombrello AK, Zavialov AV, Toro C, Zavialov AV, Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med. 2014;370(10):911\u2013920.10.1056\/NEJMoa130736124552284"},{"issue":"2","key":"b80","series-title":"J Stroke Cerebrovasc Dis","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/S1052-3057(99)80054-8","article-title":"Moyamoya syndrome associated with Sneddon\u2019s syndrome and antiphospholipid-protein antibodies","volume":"8","author":"Carhuapoma JR","year":"1999","unstructured":"Carhuapoma JR, D\u2019Olhaberriague L, Levine SR. Moyamoya syndrome associated with Sneddon\u2019s syndrome and antiphospholipid-protein antibodies. J Stroke Cerebrovasc Dis. 1999;8(2):51\u201356.10.1016\/S1052-3057(99)80054-817895140"},{"issue":"8","key":"b81","series-title":"Dev Med Child Neurol","doi-asserted-by":"publisher","first-page":"725","DOI":"10.1111\/j.1469-8749.2010.03727.x","article-title":"Intracerebral large artery disease in Aicardi-Gouti\u00e8res syndrome implicates SAMHD1 in vascular homeostasis","volume":"52","author":"Ramesh V","year":"2010","unstructured":"Ramesh V, Bernardi B, Stafa A, Garone C, Franzoni E, Abinun M, Intracerebral large artery disease in Aicardi-Gouti\u00e8res syndrome implicates SAMHD1 in vascular homeostasis. Dev Med Child Neurol. 2010;52(8):725\u2013732.2065373610.1111\/j.1469-8749.2010.03727.x"},{"issue":"7","key":"b82","series-title":"Nat Rev Immunol","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1038\/nri3850","article-title":"Aicardi-Gouti\u00e8res syndrome and the type I interferonopathies","volume":"15","author":"Crow YJ","year":"2015","unstructured":"Crow YJ, Manel N. Aicardi-Gouti\u00e8res syndrome and the type I interferonopathies. Nat Rev Immunol. 2015;15(7):429\u2013440.10.1038\/nri385026052098"},{"issue":"3","key":"b83","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1016\/j.ajhg.2014.01.018","article-title":"Loss of \u03b11\u03b21 soluble guanylate cyclase, the major nitric oxide receptor, leads to moyamoya and achalasia","volume":"94","author":"Herv\u00e9 D","year":"2014","unstructured":"Herv\u00e9 D, Philippi A, Belbouab R, Zerah M, Chabrier S, Collardeau-Frachon S, Loss of \u03b11\u03b21 soluble guanylate cyclase, the major nitric oxide receptor, leads to moyamoya and achalasia. Am J Hum Genet. 2014;94(3):385\u2013394.2458174210.1016\/j.ajhg.2014.01.018"},{"issue":"5","key":"b84","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"617","DOI":"10.1016\/j.ajhg.2009.04.007","article-title":"Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and moyamoya disease, along with thoracic aortic disease","volume":"84","author":"Guo DC","year":"2009","unstructured":"Guo DC, Papke CL, Tran-Fadulu V, Regalado ES, Avidan N, Johnson RJ, Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and moyamoya disease, along with thoracic aortic disease. Am J Hum Genet. 2009;84(5):617\u2013627.1940952510.1016\/j.ajhg.2009.04.007"},{"issue":"2","key":"b85","series-title":"Stroke","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1161\/01.STR.22.2.269","article-title":"Turner\u2019s syndrome, fibromuscular dysplasia, and stroke","volume":"22","author":"Lancman M","year":"1991","unstructured":"Lancman M, Mesropian H, Serra P, Granillo R. Turner\u2019s syndrome, fibromuscular dysplasia, and stroke. Stroke. 1991;22(2):269\u2013271.10.1161\/01.STR.22.2.2692003292"},{"key":"b86","series-title":"World Neurosurg","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1016\/j.wneu.2017.08.176","article-title":"Moyamoya vasculopathy in PHACE syndrome: six new cases and review of the literature","volume":"108","author":"Tortora D","year":"2017","unstructured":"Tortora D, Severino M, Accogli A, Martinetti C, Vercellino N, Capra V, Moyamoya vasculopathy in PHACE syndrome: six new cases and review of the literature. World Neurosurg. 2017;108:291\u2013302.10.1016\/j.wneu.2017.08.17628887276"},{"issue":"1","key":"b87","series-title":"Br J Dermatol","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1111\/bjd.12435","article-title":"Pathogenesis of infantile haemangioma","volume":"169","author":"Greenberger S","year":"2013","unstructured":"Greenberger S, Bischoff J. Pathogenesis of infantile haemangioma. Br J Dermatol. 2013;169(1):12\u201319.2366847410.1111\/bjd.12435"},{"issue":"13","key":"b88","series-title":"Invest Ophthalmol Vis Sci","first-page":"1342","article-title":"Overexpression of GLUT1 and increased glucose transport leads to apoptosis in retinal endothelial cells","volume":"43","author":"Kumagai AK","year":"2002","unstructured":"Kumagai AK, Jamali JA, Deo BK, Hosoya K, Terasaki T. Overexpression of GLUT1 and increased glucose transport leads to apoptosis in retinal endothelial cells. Invest Ophthalmol Vis Sci. 2002;43(13):1342."},{"issue":"9","key":"b89","series-title":"Arterioscler Thromb Vasc Biol","doi-asserted-by":"publisher","first-page":"1646","DOI":"10.1161\/ATVBAHA.117.309510","article-title":"Endothelial cell autophagy maintains shear stress-induced nitric oxide generation via glycolysis-dependent purinergic signaling to endothelial nitric oxide synthase","volume":"37","author":"Bharath LP","year":"2017","unstructured":"Bharath LP, Cho JM, Park SK, Ruan T, Li Y, Mueller R, Endothelial cell autophagy maintains shear stress-induced nitric oxide generation via glycolysis-dependent purinergic signaling to endothelial nitric oxide synthase. Arterioscler Thromb Vasc Biol. 2017;37(9):1646\u20131656.10.1161\/ATVBAHA.117.30951028684613"},{"issue":"1","key":"b90","series-title":"Biomol Ther (Seoul)","doi-asserted-by":"publisher","first-page":"45","DOI":"10.4062\/biomolther.2019.119","article-title":"The role of a neurovascular signaling pathway involving hypoxia-inducible factor and notch in the function of the central nervous system","volume":"28","author":"Kim S","year":"2020","unstructured":"Kim S, Lee M, Choi YK. The role of a neurovascular signaling pathway involving hypoxia-inducible factor and notch in the function of the central nervous system. Biomol Ther (Seoul). 2020;28(1):45\u201357.10.4062\/biomolther.2019.119"}],"container-title":["Neurosurgical Focus"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/neurosurg-focus\/51\/3\/article-pE6.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/neurosurg-focus\/51\/3\/article-pE6.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/neurosurg-focus\/51\/3\/article-pE6.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,6]],"date-time":"2026-06-06T08:26:51Z","timestamp":1780734411000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/neurosurg-focus\/51\/3\/article-pE6.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9]]},"references-count":180,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.3171\/2021.6.focus21281","relation":{},"ISSN":["1092-0684"],"issn-type":[{"value":"1092-0684","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,9]]}}}