{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T22:08:10Z","timestamp":1790892490423,"version":"4.1.0"},"reference-count":127,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2025,1,30]],"date-time":"2025-01-30T00:00:00Z","timestamp":1738195200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Natural Science Foundation of Hubei Province","award":["2023AFB762"],"award-info":[{"award-number":["2023AFB762"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Moyamoya disease (MMD) is a rare progressive cerebrovascular disorder characterized by the stenosis or occlusion of the terminal segments of the internal carotid arteries, leading to the development of abnormal collateral vascular networks. These networks are a compensatory mechanism for reduced blood flow to the brain. Despite extensive research, the exact etiology of MMD remains unknown, although recent studies suggest that immune system dysfunction plays a critical role in its pathogenesis. In particular, the involvement of immune cells such as T cells, macrophages, and dendritic cells has been increasingly recognized. These immune cells contribute to the inflammatory process and vascular remodeling observed in MMD patients, further complicating the disease\u2019s progression. Inflammation and immune-mediated damage to the vessel walls may accelerate the narrowing and occlusion of arteries, exacerbating ischemic events in the brain. Additionally, studies have revealed that certain genetic and environmental factors can influence immune system activation in MMD, linking these pathways to disease development. This review aims to provide a comprehensive overview of the immune mechanisms at play in MMD, focusing on how immune cells participate in vascular injury and remodeling. Understanding these immunological processes may offer new therapeutic targets to halt or reverse disease progression, potentially leading to more effective treatment strategies for MMD.<\/jats:p>","DOI":"10.3390\/brainsci15020137","type":"journal-article","created":{"date-parts":[[2025,1,30]],"date-time":"2025-01-30T11:08:23Z","timestamp":1738235303000},"page":"137","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The Role of Immune Cells in Moyamoya Disease"],"prefix":"10.3390","volume":"15","author":[{"given":"Sheng","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, Jiefang Avenue 1095, Wuhan 430030, China"},{"name":"Hubei Key Laboratory of Neural Injury and Functional Reconstruction, Huazhong University of Science and Technology, Wuhan 430030, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qian","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, Jiefang Avenue 1095, Wuhan 430030, China"},{"name":"Hubei Key Laboratory of Neural Injury and Functional Reconstruction, Huazhong University of Science and Technology, Wuhan 430030, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3801-0461","authenticated-orcid":false,"given":"Yuan","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, Jiefang Avenue 1095, Wuhan 430030, China"},{"name":"Hubei Key Laboratory of Neural Injury and Functional Reconstruction, Huazhong University of Science and Technology, Wuhan 430030, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5430-6775","authenticated-orcid":false,"given":"Xincheng","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, Jiefang Avenue 1095, Wuhan 430030, China"},{"name":"Hubei Key Laboratory of Neural Injury and Functional Reconstruction, Huazhong University of Science and Technology, Wuhan 430030, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yimin","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, Jiefang Avenue 1095, Wuhan 430030, China"},{"name":"Hubei Key Laboratory of Neural Injury and Functional Reconstruction, Huazhong University of Science and Technology, Wuhan 430030, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3023-2040","authenticated-orcid":false,"given":"Huaqiu","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, Jiefang Avenue 1095, Wuhan 430030, China"},{"name":"Hubei Key Laboratory of Neural Injury and Functional Reconstruction, Huazhong University of Science and Technology, Wuhan 430030, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,30]]},"reference":[{"key":"ref_1","first-page":"100331","article-title":"Epidemiology of moyamoya disease in china: A nationwide hospital-based study","volume":"18","author":"Zhang","year":"2022","journal-title":"Lancet Reg. Health West Pac."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1016\/S1474-4422(22)00165-X","article-title":"Moyamoya disease: Diagnosis and interventions","volume":"21","author":"Ihara","year":"2022","journal-title":"Lancet Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"307","DOI":"10.2176\/jns-nmc.2022-0072","article-title":"Research Committee on Moyamoya Disease of the Ministry of Health L, Welfare J. Diagnostic Criteria for Moyamoya Disease\u20142021 Revised Version","volume":"62","author":"Kuroda","year":"2022","journal-title":"Neurol. Med. Chir."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1038\/jhg.2010.132","article-title":"A genome-wide association study identifies RNF213 as the first Moyamoya disease gene","volume":"56","author":"Kamada","year":"2011","journal-title":"J. Hum. Genet."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Mineharu, Y., and Miyamoto, S. (2021). RNF213 and GUCY1A3 in Moyamoya Disease: Key Regulators of Metabolism, Inflammation, and Vascular Stability. Front. Neurol., 12.","DOI":"10.3389\/fneur.2021.687088"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2107","DOI":"10.1177\/0271678X221110679","article-title":"RNF213 loss of function reshapes vascular transcriptome and spliceosome leading to disrupted angiogenesis and aggravated vascular inflammatory responses","volume":"42","author":"Zhang","year":"2022","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1358","DOI":"10.1161\/01.STR.22.11.1358","article-title":"Systemic vascular changes in spontaneous occlusion of the circle of Willis","volume":"22","author":"Ikeda","year":"1991","journal-title":"Stroke"},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"Morimoto, T., Mineharu, Y., Ono, K., Nakatochi, M., Ichihara, S., Kabata, R., Takagi, Y., Cao, Y., Zhao, L., and Kobayashi, H. (2017). Significant association of RNF213 p.R4810K, a moyamoya susceptibility variant, with coronary artery disease. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0175649"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1038\/s41588-020-0705-3","article-title":"Population-specific and trans-ancestry genome-wide analyses identify distinct and shared genetic risk loci for coronary artery disease","volume":"52","author":"Koyama","year":"2020","journal-title":"Nat. Genet."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e2218825120","DOI":"10.1073\/pnas.2218825120","article-title":"RNF213 modulates gamma-herpesvirus infection and reactivation via targeting the viral Replication and Transcription Activator","volume":"120","author":"Tian","year":"2023","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1038\/s41586-021-03566-4","article-title":"Ubiquitylation of lipopolysaccharide by RNF213 during bacterial infection","volume":"594","author":"Otten","year":"2021","journal-title":"Nature"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1177\/1747493017694393","article-title":"Etiology and pathogenesis of Moyamoya Disease: An update on disease prevalence","volume":"12","author":"Huang","year":"2017","journal-title":"Int. J. Stroke"},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Pollaci, G., Gorla, G., Potenza, A., Carrozzini, T., Canavero, I., Bersano, A., and Gatti, L. (2022). Novel Multifaceted Roles for RNF213 Protein. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23094492"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2552","DOI":"10.1097\/CM9.0000000000002985","article-title":"RNF213 in moyamoya disease: Genotype-phenotype association and the underlying mechanism","volume":"137","author":"Fang","year":"2024","journal-title":"Chin. Med. J."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1016\/j.molmed.2022.08.009","article-title":"Moyamoya disease emerging as an immune-related angiopathy","volume":"28","author":"Asselman","year":"2022","journal-title":"Trends Mol. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e1647","DOI":"10.1002\/ctm2.1647","article-title":"Circulating immune cell landscape and T-cell abnormalities in patients with moyamoya disease","volume":"14","author":"Ge","year":"2024","journal-title":"Clin. Transl. Med."},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Zhou, Z., Wang, Y., Zhang, J., Liu, Z., Hao, X., Wang, X., He, S., and Wang, R. (2024). Characterization of PANoptosis-related genes and the immune landscape in moyamoya disease. Sci. Rep., 14.","DOI":"10.1038\/s41598-024-61241-w"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"5961","DOI":"10.1038\/s41467-024-50392-z","article-title":"RNF213 promotes Treg cell differentiation by facilitating K63-linked ubiquitination and nuclear translocation of FOXO1","volume":"15","author":"Yang","year":"2024","journal-title":"Nat. Commun."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Liu, E., Liu, C., Jin, L., Zhou, H., Tan, X., Zhang, G., Tao, W., Gao, X., Zhao, H., and Luo, C. (2023). Clinical value of the systemic immune-inflammation index in moyamoya disease. Front. Neurol., 14.","DOI":"10.3389\/fneur.2023.1123951"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1186\/s13023-022-02238-4","article-title":"Identification of immune-infiltrated hub genes as potential biomarkers of Moyamoya disease by bioinformatics analysis","volume":"17","author":"Jin","year":"2022","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1056\/NEJMoa1701719","article-title":"Clonal Hematopoiesis and Risk of Atherosclerotic Cardiovascular Disease","volume":"377","author":"Jaiswal","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1161\/CIRCRESAHA.118.311378","article-title":"Mechanisms of Vascular Aging","volume":"123","author":"Ungvari","year":"2018","journal-title":"Circ. Res."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/0047-6374(94)90025-6","article-title":"Human arterial smooth muscle cell strains derived from patients with moyamoya disease: Changes in biological characteristics and proliferative response during cellular aging in vitro","volume":"75","author":"Fukai","year":"1994","journal-title":"Mech. Ageing Dev."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1437","DOI":"10.1161\/CIRCRESAHA.120.316770","article-title":"Microanatomy of the Human Atherosclerotic Plaque by Single-Cell Transcriptomics","volume":"127","author":"Depuydt","year":"2020","journal-title":"Circ. Res."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1111\/j.1742-7843.2011.00760.x","article-title":"Immune modulation of resistance artery remodelling","volume":"110","author":"Schiffrin","year":"2012","journal-title":"Basic Clin. Pharmacol. Toxicol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1038\/s41392-023-01451-2","article-title":"Immunosenescence: Molecular mechanisms and diseases","volume":"8","author":"Liu","year":"2023","journal-title":"Signal. Transduct. Target. Ther."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1038\/s43587-021-00082-y","article-title":"An inflammatory aging clock (iAge) based on deep learning tracks multimorbidity, immunosenescence, frailty and cardiovascular aging","volume":"1","author":"Sayed","year":"2021","journal-title":"Nat. Aging"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"3350","DOI":"10.1111\/febs.15327","article-title":"Glycolysis\u2014A key player in the inflammatory response","volume":"287","author":"Oller","year":"2020","journal-title":"FEBS J."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1379","DOI":"10.1016\/j.exger.2013.09.003","article-title":"The Th17\/Treg balance is disturbed during aging","volume":"48","author":"Schmitt","year":"2013","journal-title":"Exp. Gerontol."},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Shirakawa, K., and Sano, M. (2021). T Cell Immunosenescence in Aging, Obesity, and Cardiovascular Disease. Cells, 10.","DOI":"10.3390\/cells10092435"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/S0070-2153(06)79005-6","article-title":"Elastin-elastases and inflamm-aging","volume":"79","author":"Antonicelli","year":"2007","journal-title":"Curr. Top Dev. Biol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"818","DOI":"10.1093\/ibd\/izac199","article-title":"The Pathogenicity and Synergistic Action of Th1 and Th17 Cells in Inflammatory Bowel Diseases","volume":"29","author":"Cao","year":"2023","journal-title":"Inflamm. Bowel Dis."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1016\/j.immuni.2009.05.001","article-title":"Plasticity of CD4+ T cell lineage differentiation","volume":"30","author":"Zhou","year":"2009","journal-title":"Immunity"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1146\/annurev.immunol.021908.132710","article-title":"IL-17 and Th17 Cells","volume":"27","author":"Korn","year":"2009","journal-title":"Annu. Rev. Immunol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"2280991","DOI":"10.1080\/07853890.2023.2280991","article-title":"Study of cytokine-induced immunity in bullous pemphigoid: Recent developments","volume":"55","author":"Huang","year":"2023","journal-title":"Ann. Med."},{"key":"ref_36","doi-asserted-by":"crossref","unstructured":"Jin, K., Parreau, S., Warrington, K.J., Koster, M.J., Berry, G.J., Goronzy, J.J., and Weyand, C.M. (2022). Regulatory T Cells in Autoimmune Vasculitis. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.844300"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1038\/s41422-020-0324-7","article-title":"Transcriptional and epigenetic basis of Treg cell development and function: Its genetic anomalies or variations in autoimmune diseases","volume":"30","author":"Ohkura","year":"2020","journal-title":"Cell Res."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"e158450","DOI":"10.1172\/JCI158450","article-title":"Cellular senescence: A key therapeutic target in aging and diseases","volume":"132","author":"Zhang","year":"2022","journal-title":"J. Clin. Investig."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"111428","DOI":"10.1016\/j.mad.2020.111428","article-title":"Effects of cellular senescence on metabolic pathways in non-immune and immune cells","volume":"194","author":"Frasca","year":"2021","journal-title":"Mech. Ageing Dev."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1802","DOI":"10.1016\/j.bbadis.2018.08.008","article-title":"Endothelial cell senescence in aging-related vascular dysfunction","volume":"1865","author":"Jia","year":"2019","journal-title":"Biochim. Biophys. Acta Mol. Basis Dis."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1161\/CIRCRESAHA.121.318052","article-title":"Hypertension: Do Inflammation and Immunity Hold the Key to Solving this Epidemic?","volume":"128","author":"Madhur","year":"2021","journal-title":"Circ. Res."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1042\/CS20130407","article-title":"Immune mechanisms in hypertension and vascular injury","volume":"126","author":"Schiffrin","year":"2014","journal-title":"Clin. Sci. (Lond.)"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1007\/s11886-008-0073-6","article-title":"Is hypertension an immunologic disease?","volume":"10","author":"Harrison","year":"2008","journal-title":"Curr. Cardiol. Rep."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1093\/eurheartj\/ehu119","article-title":"Dual opposing roles of adaptive immunity in hypertension","volume":"35","author":"Mian","year":"2014","journal-title":"Eur. Heart J."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1430","DOI":"10.1161\/HYPERTENSIONAHA.112.199265","article-title":"Interferon-gamma signaling inhibition ameliorates angiotensin II-induced cardiac damage","volume":"60","author":"Marko","year":"2012","journal-title":"Hypertension"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1821","DOI":"10.1096\/fj.11-195198","article-title":"Interferon-gamma biphasically regulates angiotensinogen expression via a JAK-STAT pathway and suppressor of cytokine signaling 1 (SOCS1) in renal proximal tubular cells","volume":"26","author":"Satou","year":"2012","journal-title":"FASEB J."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1853","DOI":"10.1111\/bph.14359","article-title":"Salt, inflammation, IL-17 and hypertension","volume":"176","author":"Wenzel","year":"2019","journal-title":"Br. J. Pharmacol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1038\/s41569-023-00964-1","article-title":"Immune and inflammatory mechanisms in hypertension","volume":"21","author":"Guzik","year":"2024","journal-title":"Nat. Rev. Cardiol."},{"key":"ref_49","first-page":"132","article-title":"Th17\/Treg imbalance in patients with primary hyperaldosteronism and resistant hypertension","volume":"132","author":"Imiela","year":"2022","journal-title":"Pol. Arch. Intern. Med."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1038\/nrcardio.2015.169","article-title":"Regulatory T cells in cardiovascular diseases","volume":"13","author":"Meng","year":"2016","journal-title":"Nat. Rev. Cardiol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"30","DOI":"10.2165\/00003495-199700531-00006","article-title":"Endothelial dysfunction and hypertension","volume":"53","author":"Ferro","year":"1997","journal-title":"Drugs"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1007\/5584_2016_90","article-title":"Endothelial dysfunction and hypertension","volume":"956","author":"Konukoglu","year":"2017","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1093\/cvr\/cvn022","article-title":"Hypertension and microvascular remodelling","volume":"78","author":"Feihl","year":"2008","journal-title":"Cardiovasc. Res."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.biopha.2017.07.091","article-title":"Renin-angiotensin-aldosterone (raas): The ubiquitous system for homeostasis and pathologies","volume":"94","author":"Patel","year":"2017","journal-title":"Biomed. Pharmacother."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"2449","DOI":"10.1084\/jem.20070657","article-title":"Role of the T cell in the genesis of angiotensin II induced hypertension and vascular dysfunction","volume":"204","author":"Guzik","year":"2007","journal-title":"J. Exp. Med."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"e125527","DOI":"10.1172\/jci.insight.125527","article-title":"CXCR3 regulates CD4+ T cell cardiotropism in pressure overload-induced cardiac dysfunction","volume":"4","author":"Ngwenyama","year":"2019","journal-title":"JCI Insight"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1161\/HYPERTENSIONAHA.110.163576","article-title":"Inflammation, immunity, and hypertension","volume":"57","author":"Harrison","year":"2011","journal-title":"Hypertension"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1161\/01.HYP.0000075082.06183.4E","article-title":"Imbalance of T-cell subsets in angiotensin II-infused hypertensive rats with kidney injury","volume":"42","author":"Shao","year":"2003","journal-title":"Hypertension"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1960","DOI":"10.1161\/01.STR.24.12.1960","article-title":"Smooth muscle cell proliferation and localization of macrophages and T cells in the occlusive intracranial major arteries in moyamoya disease","volume":"24","author":"Masuda","year":"1993","journal-title":"Stroke"},{"key":"ref_60","doi-asserted-by":"crossref","unstructured":"Li, S., Han, Y., Zhang, Q., Tang, D., Li, J., and Weng, L. (2022). Comprehensive molecular analyses of an autoimmune-related gene predictive model and immune infiltrations using machine learning methods in moyamoya disease. Front. Mol. Biosci., 9.","DOI":"10.3389\/fmolb.2022.991425"},{"key":"ref_61","doi-asserted-by":"crossref","unstructured":"Cao, L., Ai, Y., Dong, Y., Li, D., Wang, H., Sun, K., Wang, C., Zhang, M., Yan, D., and Li, H. (2023). Bioinformatics analysis reveals the landscape of immune cell infiltration and novel immune-related biomarkers in moyamoya disease. Front. Genet., 14.","DOI":"10.3389\/fgene.2023.1101612"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"e84","DOI":"10.1161\/STROKEAHA.113.002228","article-title":"Regulatory T cells in ischemic stroke: Helpful or hazardous?","volume":"44","author":"Schabitz","year":"2013","journal-title":"Stroke"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"e3328","DOI":"10.1002\/brb3.3328","article-title":"Proteomic and metabolomic characterizations of moyamoya disease patient sera","volume":"13","author":"Guo","year":"2023","journal-title":"Brain Behav."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1159\/000353557","article-title":"Regulatory T cells, leptin and angiogenesis","volume":"99","author":"Pucino","year":"2014","journal-title":"Chem. Immunol. Allergy"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"E3","DOI":"10.3171\/2021.6.FOCUS20870","article-title":"Transcriptome-wide analysis of intracranial artery in patients with moyamoya disease showing upregulation of immune response, and downregulation of oxidative phosphorylation and DNA repair","volume":"51","author":"Kanamori","year":"2021","journal-title":"Neurosurg. Focus"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"e209658","DOI":"10.1212\/WNL.0000000000209658","article-title":"Clinical Course, Therapy, and Long-Term Outcomes of Children with Moyamoya Disease and Posterior Cerebral Artery Involvement","volume":"103","author":"Wang","year":"2024","journal-title":"Neurology"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1007\/s12975-023-01135-7","article-title":"Comprehensive Profiling of Secreted Factors in the Cerebrospinal Fluid of Moyamoya Disease Patients","volume":"15","author":"Abhinav","year":"2024","journal-title":"Transl. Stroke Res."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.12998\/wjcc.v12.i10.1830","article-title":"Moyamoya syndrome may result from psoriasis: Four case reports","volume":"12","author":"Chen","year":"2024","journal-title":"World J. Clin. Cases"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"145340","DOI":"10.1016\/j.gene.2020.145340","article-title":"New insights into TNFalpha\/PTP1B and PPARgamma pathway through RNF213- a link between inflammation, obesity, insulin resistance, and Moyamoya disease","volume":"771","author":"Sarkar","year":"2021","journal-title":"Gene"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1002\/iub.1344","article-title":"Inflammation in arterial diseases","volume":"67","author":"Carbone","year":"2015","journal-title":"IUBMB Life"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1038\/s41584-018-0145-y","article-title":"Pathogenesis and therapeutic interventions for ANCA-associated vasculitis","volume":"15","author":"Nakazawa","year":"2019","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.mri.2021.08.004","article-title":"Intracranial vessel wall imaging framework\u2014Data acquisition, processing, and visualization","volume":"83","author":"Guggenberger","year":"2021","journal-title":"Magn. Reson. Imaging"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1097\/BOR.0000000000000759","article-title":"Intracranial vessel wall imaging","volume":"33","author":"Arslan","year":"2021","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"C7","DOI":"10.1016\/j.jacc.2005.09.068","article-title":"Pathogenesis of atherosclerosis","volume":"47","author":"Falk","year":"2006","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"102813","DOI":"10.1016\/j.jaut.2022.102813","article-title":"Cardiovascular disease risk in antiphospholipid syndrome: Thrombo-inflammation and atherothrombosis","volume":"128","author":"Tektonidou","year":"2022","journal-title":"J. Autoimmun."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1038\/s41577-019-0160-5","article-title":"Immune mechanisms of hypertension","volume":"19","author":"Drummond","year":"2019","journal-title":"Nat. Rev. Immunol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"111255","DOI":"10.1016\/j.mce.2021.111255","article-title":"Advances in use of mouse models to study the renin-angiotensin system","volume":"529","author":"Emathinger","year":"2021","journal-title":"Mol. Cell Endocrinol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"S511","DOI":"10.1097\/00005344-199100177-00146","article-title":"Acute and chronic effects of anti-endothelin-1 antibody on blood pressure in spontaneously hypertensive rats","volume":"17","author":"Kinoshita","year":"1991","journal-title":"J. Cardiovasc. Pharmacol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1007\/s00281-022-00920-0","article-title":"Mechanisms of vascular damage in ANCA vasculitis","volume":"44","author":"Herrera","year":"2022","journal-title":"Semin. Immunopathol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1007\/s00281-022-00916-w","article-title":"Mechanisms of immunothrombosis and vasculopathy in antiphospholipid syndrome","volume":"44","author":"Knight","year":"2022","journal-title":"Semin. Immunopathol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/ijo.2017.260","article-title":"Serum oxldl-beta2gpi complex reflects metabolic syndrome and inflammation in adipose tissue in obese","volume":"42","author":"Siklova","year":"2018","journal-title":"Int. J. Obes. (Lond.)"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1113\/expphysiol.2004.028456","article-title":"Polymorphism in angiotensin ii receptor genes and hypertension","volume":"90","author":"Baudin","year":"2005","journal-title":"Exp. Physiol."},{"key":"ref_83","first-page":"556","article-title":"Systemic vasculitis","volume":"83","author":"Sharma","year":"2011","journal-title":"Am. Fam. Physician"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"830","DOI":"10.1161\/CIRCRESAHA.111.248542","article-title":"B1a b lymphocytes are atheroprotective by secreting natural igm that increases igm deposits and reduces necrotic cores in atherosclerotic lesions","volume":"109","author":"Kyaw","year":"2011","journal-title":"Circ. Res."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"e002947","DOI":"10.1161\/JAHA.115.002947","article-title":"Toll-Like Receptor (TLR)4 and MyD88 are Essential for Atheroprotection by Peritoneal B1a B Cells","volume":"5","author":"Hosseini","year":"2016","journal-title":"J. Am. Heart Assoc."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"4410","DOI":"10.4049\/jimmunol.1000033","article-title":"Conventional B2 B cell depletion ameliorates whereas its adoptive transfer aggravates atherosclerosis","volume":"185","author":"Kyaw","year":"2010","journal-title":"J. Immunol."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.4049\/jimmunol.167.2.1081","article-title":"Lipopolysaccharide-activated B cells down-regulate Th1 immunity and prevent autoimmune diabetes in nonobese diabetic mice","volume":"167","author":"Tian","year":"2001","journal-title":"J. Immunol."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1038\/nm.3554","article-title":"Interleukin-35 induces regulatory B cells that suppress autoimmune disease","volume":"20","author":"Wang","year":"2014","journal-title":"Nat. Med."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1782","DOI":"10.1093\/eurheartj\/ehu203","article-title":"Anti-inflammatory therapies for cardiovascular disease","volume":"35","author":"Ridker","year":"2014","journal-title":"Eur. Heart J."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1016\/j.wneu.2017.01.012","article-title":"Moyamoya and Inflammation","volume":"100","author":"Ellis","year":"2017","journal-title":"World Neurosurg."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"43","DOI":"10.2176\/jns-nmc.2023-0169","article-title":"Synergistic Interaction of Thyroid Autoantibodies and Ring Finger Protein 213 Variant in Moyamoya Disease","volume":"64","author":"Thamamongood","year":"2024","journal-title":"Neurol. Med. Chir."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1111\/ijs.12177","article-title":"Elevated thyroid autoantibodies and intracranial stenosis in stroke at an early age","volume":"9","author":"Shi","year":"2014","journal-title":"Int. J. Stroke"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1007\/s00424-017-1941-y","article-title":"Macrophages in vascular inflammation and atherosclerosis","volume":"469","author":"Cochain","year":"2017","journal-title":"Pflug. Arch."},{"key":"ref_94","first-page":"970890","article-title":"Angiogenesis in Inflammatory Bowel Disease","volume":"2015","author":"Alkim","year":"2015","journal-title":"Int. J. Inflam."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"578","DOI":"10.3748\/wjg.v17.i5.578","article-title":"Role of the endothelium in inflammatory bowel diseases","volume":"17","author":"Cromer","year":"2011","journal-title":"World J. Gastroenterol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.brainres.2017.11.013","article-title":"Increased serum production of soluble CD163 and CXCL5 in patients with moyamoya disease: Involvement of intrinsic immune reaction in its pathogenesis","volume":"1679","author":"Fujimura","year":"2018","journal-title":"Brain Res."},{"key":"ref_97","doi-asserted-by":"crossref","unstructured":"Nagata, E., Masuda, H., Nakayama, T., Netsu, S., Yuzawa, H., Fujii, N., Kohara, S., Sorimachi, T., Osada, T., and Imazeki, R. (2019). Insufficient production of IL-10 from M2 macrophages impairs in vitro endothelial progenitor cell differentiation in patients with Moyamoya disease. Sci. Rep., 9.","DOI":"10.1038\/s41598-019-53114-4"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"H368","DOI":"10.1152\/ajpheart.00603.2016","article-title":"Dendritic cells and isolevuglandins in immunity, inflammation, and hypertension","volume":"312","author":"Dixon","year":"2017","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"7554","DOI":"10.1002\/jcp.30396","article-title":"Dysregulation of Rnf 213 gene contributes to T cell response via antigen uptake, processing, and presentation","volume":"236","author":"Tashiro","year":"2021","journal-title":"J. Cell Physiol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1007\/s12026-024-09464-x","article-title":"Mass cytometry revealed the circulating immune cell landscape across different Suzuki stages of Moyamoya disease","volume":"72","author":"Liu","year":"2024","journal-title":"Immunol. Res."},{"key":"ref_101","doi-asserted-by":"crossref","unstructured":"Shirozu, N., Ohgidani, M., Hata, N., Tanaka, S., Inamine, S., Sagata, N., Kimura, T., Inoue, I., Arimura, K., and Nakamizo, A. (2023). Angiogenic and inflammatory responses in human induced microglia-like (iMG) cells from patients with Moyamoya disease. Sci. Rep., 13.","DOI":"10.1038\/s41598-023-41456-z"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"1721","DOI":"10.2147\/JIR.S451538","article-title":"High Level of Serum Complement C3 Expression is Associated with Postoperative Vasculopathy Progression in Moyamoya Disease","volume":"17","author":"Wang","year":"2024","journal-title":"J. Inflamm. Res."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1007\/s12264-023-01158-y","article-title":"GM-CSF Promotes the Development of Dysfunctional Vascular Networks in Moyamoya Disease","volume":"40","author":"Li","year":"2024","journal-title":"Neurosci. Bull."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"1008","DOI":"10.1166\/jnn.2021.18682","article-title":"Study on novel nanoparticle slow-release drugs for moyamoya disease","volume":"21","author":"Qiu","year":"2021","journal-title":"J. Nanosci. Nanotechnol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1973","DOI":"10.1161\/STROKEAHA.119.024689","article-title":"Characteristics of moyamoya disease based on national registry data in japan","volume":"50","author":"Sato","year":"2019","journal-title":"Stroke"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1080\/01616412.2019.1580455","article-title":"Cilostazol may improve cognition better than clopidogrel in non-surgical adult patients with ischemic moyamoya disease: Subanalysis of a prospective cohort","volume":"41","author":"Ando","year":"2019","journal-title":"Neurol. Res."},{"key":"ref_107","doi-asserted-by":"crossref","unstructured":"Liu, Z., He, S., Xu, Z., Duan, R., Yuan, L., Xiao, C., Yi, Z., and Wang, R. (2020). Association between white matter impairment and cognitive dysfunction in patients with ischemic moyamoya disease. BMC Neurol., 20.","DOI":"10.1186\/s12883-020-01876-0"},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1007\/s40263-017-0459-3","article-title":"Pharmacotherapy for vascular cognitive impairment","volume":"31","author":"Farooq","year":"2017","journal-title":"CNS Drugs"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"e928","DOI":"10.1016\/j.wneu.2017.11.113","article-title":"Effectiveness of ipsilateral stroke prevention between conservative management and indirect revascularization for moyamoya disease in a north american cohort","volume":"110","author":"Porras","year":"2018","journal-title":"World Neurosurg."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"2305","DOI":"10.1007\/s10143-022-01749-9","article-title":"Impact of rnf213 founder polymorphism (p.R4810k) on the postoperative development of indirect pial synangiosis after direct\/indirect combined revascularization surgery for adult moyamoya disease","volume":"45","author":"Ito","year":"2022","journal-title":"Neurosurg. Rev."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"1801","DOI":"10.1177\/0271678X241245557","article-title":"Rnf213 variant and autophagic impairment: A pivotal link to endothelial dysfunction in moyamoya disease","volume":"44","author":"Shin","year":"2024","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"4674","DOI":"10.1093\/brain\/awad225","article-title":"Rnf213 loss-of-function promotes pathological angiogenesis in moyamoya disease via the hippo pathway","volume":"146","author":"Ye","year":"2023","journal-title":"Brain"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"2191","DOI":"10.1161\/ATVBAHA.116.307559","article-title":"B-cell depletion promotes aortic infiltration of immunosuppressive cells and is protective of experimental aortic aneurysm","volume":"36","author":"Schaheen","year":"2016","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"6308","DOI":"10.1111\/jcmm.14517","article-title":"Early restoration of immune and vascular phenotypes in systemic lupus erythematosus and rheumatoid arthritis patients after b cell depletion","volume":"23","author":"Cecchi","year":"2019","journal-title":"J. Cell Mol. Med."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1038\/s41584-022-00770-y","article-title":"Endothelial function and endothelial progenitor cells in systemic lupus erythematosus","volume":"18","author":"Mak","year":"2022","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"2317","DOI":"10.1016\/j.jacc.2017.02.058","article-title":"Antiphospholipid syndrome: Role of vascular endothelial cells and implications for risk stratification and targeted therapeutics","volume":"69","author":"Corban","year":"2017","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_117","doi-asserted-by":"crossref","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","year":"2010","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_118","doi-asserted-by":"crossref","unstructured":"Liu, W., Sun, J., Shi, Z., Huang, Z., Yu, L., Du, H., Ge, P., and Zhang, D. (2023). Circulating inflammatory cytokine associated with poor prognosis in moyamoya disease: A prospective cohort study. J. Clin. Med., 12.","DOI":"10.3390\/jcm12030823"},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"2247","DOI":"10.1007\/s00204-024-03751-0","article-title":"Vascular endothelial dysfunction induced by 3-bromofluoranthene via mapk-mediated-nfkappab pro-inflammatory pathway and intracellular ros generation","volume":"98","author":"Lee","year":"2024","journal-title":"Arch. Toxicol."},{"key":"ref_120","doi-asserted-by":"crossref","unstructured":"Dong, X., Wu, D., Zhang, Y., Jia, L., Pan, X., Sun, J., and Pan, L.L. (2020). Cathelicidin modulates vascular smooth muscle cell phenotypic switching through ros\/il-6 pathway. Antioxidants, 9.","DOI":"10.3390\/antiox9060491"},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"1705","DOI":"10.3171\/2023.4.JNS23479","article-title":"Assessment of plasma soluble tie-2 level to distinguish moyamoya disease from atherosclerotic cerebrovascular disease and predict postoperative neovascularization","volume":"139","author":"Chen","year":"2023","journal-title":"J. Neurosurg."},{"key":"ref_122","doi-asserted-by":"crossref","unstructured":"Dabravolski, S.A., Khotina, V.A., Omelchenko, A.V., Kalmykov, V.A., and Orekhov, A.N. (2022). The role of the vegf family in atherosclerosis development and its potential as treatment targets. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23020931"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1016\/j.ebiom.2018.06.003","article-title":"Il-33 initiates vascular remodelling in hypoxic pulmonary hypertension by up-regulating hif-1alpha and vegf expression in vascular endothelial cells","volume":"33","author":"Liu","year":"2018","journal-title":"EBioMedicine"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1007\/s12975-019-00743-6","article-title":"Moyamoya disease and spectrums of rnf213 vasculopathy","volume":"11","author":"Bang","year":"2020","journal-title":"Transl. Stroke Res."},{"key":"ref_125","doi-asserted-by":"crossref","unstructured":"Hiraide, T., Suzuki, H., Momoi, M., Shinya, Y., Fukuda, K., Kosaki, K., and Kataoka, M. (2022). Rnf213-associated vascular disease: A concept unifying various vasculopathies. Life, 12.","DOI":"10.3390\/life12040555"},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"1263","DOI":"10.1161\/STROKEAHA.120.032691","article-title":"Moyamoya disease susceptibility gene rnf213 regulates endothelial barrier function","volume":"53","author":"Roy","year":"2022","journal-title":"Stroke"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"102074","DOI":"10.1016\/j.labinv.2024.102074","article-title":"Identification of rnf213 as a potential suppressor of local invasion in intrahepatic cholangiocarcinoma","volume":"104","author":"Chiablaem","year":"2024","journal-title":"Lab. Investig."}],"container-title":["Brain Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3425\/15\/2\/137\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T10:38:58Z","timestamp":1759919938000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3425\/15\/2\/137"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,30]]},"references-count":127,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2025,2]]}},"alternative-id":["brainsci15020137"],"URL":"https:\/\/doi.org\/10.3390\/brainsci15020137","relation":{},"ISSN":["2076-3425"],"issn-type":[{"value":"2076-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,30]]}}}