{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T15:41:39Z","timestamp":1779291699940,"version":"3.51.4"},"reference-count":93,"publisher":"Elsevier BV","issue":"7","license":[{"start":{"date-parts":[[2024,7,1]],"date-time":"2024-07-01T00:00:00Z","timestamp":1719792000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,7,1]],"date-time":"2024-07-01T00:00:00Z","timestamp":1719792000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,6,4]],"date-time":"2024-06-04T00:00:00Z","timestamp":1717459200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["HL161094"],"award-info":[{"award-number":["HL161094"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008065","name":"Georgia Research Alliance","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["jbc.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Biological Chemistry"],"published-print":{"date-parts":[[2024,7]]},"DOI":"10.1016\/j.jbc.2024.107445","type":"journal-article","created":{"date-parts":[[2024,6,5]],"date-time":"2024-06-05T07:09:57Z","timestamp":1717571397000},"page":"107445","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":7,"title":["Poglut2\/3 double knockout in mice results in neonatal lethality with reduced levels of fibrillin in lung tissues"],"prefix":"10.1016","volume":"300","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2051-9865","authenticated-orcid":false,"given":"Sanjiv","family":"Neupane","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel B.","family":"Williamson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robyn A.","family":"Roth","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4889-8131","authenticated-orcid":false,"given":"Carmen M.","family":"Halabi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert S.","family":"Haltiwanger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4713-9264","authenticated-orcid":false,"given":"Bernadette C.","family":"Holdener","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.jbc.2024.107445_bib1","doi-asserted-by":"crossref","first-page":"50795","DOI":"10.1074\/jbc.M210611200","article-title":"Homo- and heterotypic fibrillin-1 and -2 interactions constitute the basis for the assembly of microfibrils","volume":"277","author":"Lin","year":"2002","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib2","doi-asserted-by":"crossref","DOI":"10.1242\/dmm.037283","article-title":"Targeted deletion of fibrillin-1 in the mouse eye results in ectopia lentis and other ocular phenotypes associated with Marfan syndrome","volume":"12","author":"Jones","year":"2019","journal-title":"Dis. Model Mech."},{"key":"10.1016\/j.jbc.2024.107445_bib3","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1016\/j.matbio.2018.01.005","article-title":"Elastin in lung development and disease pathogenesis","volume":"73","author":"Mecham","year":"2018","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib4","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1111\/iep.12239","article-title":"Fell-Muir Lecture: fibrillin microfibrils: structural tensometers of elastic tissues?","volume":"98","author":"Kielty","year":"2017","journal-title":"Int. J. Exp. Pathol."},{"key":"10.1016\/j.jbc.2024.107445_bib5","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/j.matbio.2014.02.004","article-title":"Fibulin-4 and fibulin-5 in elastogenesis and beyond: insights from mouse and human studies","volume":"37","author":"Papke","year":"2014","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib6","doi-asserted-by":"crossref","first-page":"24553","DOI":"10.1074\/jbc.M109.019364","article-title":"Differential regulation of elastic fiber formation by fibulin-4 and -5","volume":"284","author":"Choudhury","year":"2009","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib7","doi-asserted-by":"crossref","first-page":"5672","DOI":"10.1093\/hmg\/ddu283","article-title":"Latent TGF-beta binding protein-2 is essential for the development of ciliary zonule microfibrils","volume":"23","author":"Inoue","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.jbc.2024.107445_bib8","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/j.semcdb.2018.07.016","article-title":"Fibrillin microfibrils and elastic fibre proteins: functional interactions and extracellular regulation of growth factors","volume":"89","author":"Thomson","year":"2019","journal-title":"Semin. Cell Dev. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib9","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1002\/dvdy.331","article-title":"The role of LTBPs in TGF beta signaling","volume":"251","author":"Rifkin","year":"2022","journal-title":"Dev. Dyn."},{"key":"10.1016\/j.jbc.2024.107445_bib10","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1007\/978-3-030-80614-9_8","article-title":"Pathophysiology and pathogenesis of marfan syndrome","volume":"1348","author":"Zeigler","year":"2021","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib11","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1007\/978-3-030-80614-9_7","article-title":"Connective tissue disorders and cardiovascular Complications: the Indomitable role of transforming growth factor-beta signaling","volume":"1348","author":"Wheeler","year":"2021","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib12","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1111\/nyas.14465","article-title":"Acromelic dysplasias: how rare musculoskeletal disorders reveal biological functions of extracellular matrix proteins","volume":"1490","author":"Stanley","year":"2021","journal-title":"Ann. N. Y Acad. Sci."},{"key":"10.1016\/j.jbc.2024.107445_bib13","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1002\/humu.24383","article-title":"The fibrillinopathies: new insights with focus on the paradigm of opposing phenotypes for both FBN1 and FBN2","volume":"43","author":"Peeters","year":"2022","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.jbc.2024.107445_bib14","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbc.2021.101055","article-title":"POGLUT2 and POGLUT3 O-glucosylate multiple EGF repeats in fibrillin-1, -2, and LTBP1 and promote secretion of fibrillin-1","volume":"297","author":"Williamson","year":"2021","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib15","doi-asserted-by":"crossref","first-page":"E8395","DOI":"10.1073\/pnas.1804005115","article-title":"Two novel protein O-glucosyltransferases that modify sites distinct from POGLUT1 and affect Notch trafficking and signaling","volume":"115","author":"Takeuchi","year":"2018","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2024.107445_bib16","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1016\/j.str.2017.06.003","article-title":"The N-terminal region of fibrillin-1 Mediates a Bipartite interaction with LTBP1","volume":"25","author":"Robertson","year":"2017","journal-title":"Structure"},{"key":"10.1016\/j.jbc.2024.107445_bib17","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1016\/j.str.2011.12.008","article-title":"Dissecting the fibrillin microfibril: structural insights into organization and function","volume":"20","author":"Jensen","year":"2012","journal-title":"Structure"},{"key":"10.1016\/j.jbc.2024.107445_bib18","doi-asserted-by":"crossref","first-page":"5013","DOI":"10.1074\/jbc.M409029200","article-title":"Homotypic fibrillin-1 interactions in microfibril assembly","volume":"280","author":"Marson","year":"2005","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib19","doi-asserted-by":"crossref","first-page":"15841","DOI":"10.1021\/bi971036f","article-title":"Alignment of fibrillin Molecules in elastic microfibrils is defined by transglutaminase-Derived cross-Links","volume":"36","author":"Qian","year":"1997","journal-title":"Biochemistry"},{"key":"10.1016\/j.jbc.2024.107445_bib20","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1016\/j.devcel.2016.12.013","article-title":"Deciphering the Fringe-mediated Notch Code: identification of activating and Inhibiting sites Allowing Discrimination between ligands","volume":"40","author":"Kakuda","year":"2017","journal-title":"Dev. Cell"},{"key":"10.1016\/j.jbc.2024.107445_bib21","doi-asserted-by":"crossref","first-page":"15964","DOI":"10.1074\/jbc.M117.800102","article-title":"O-Glycosylation modulates the stability of epidermal growth factor-like repeats and thereby regulates Notch trafficking","volume":"292","author":"Takeuchi","year":"2017","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib22","doi-asserted-by":"crossref","first-page":"31623","DOI":"10.1074\/jbc.M111.268243","article-title":"O-glucose trisaccharide is present at high but variable stoichiometry at multiple sites on mouse Notch1","volume":"286","author":"Rana","year":"2011","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib23","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.matbio.2017.11.014","article-title":"LTBPs in biology and medicine: LTBP diseases","volume":"71-72","author":"Rifkin","year":"2018","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib24","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1002\/jcp.24704","article-title":"Function of latent TGFbeta binding protein 4 and fibulin 5 in elastogenesis and lung development","volume":"230","author":"Dabovic","year":"2015","journal-title":"J.\u00a0Cell Physiol."},{"key":"10.1016\/j.jbc.2024.107445_bib25","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1161\/ATVBAHA.122.318802","article-title":"IL11 (Interleukin-11) causes emphysematous lung disease in a mouse model of marfan syndrome","volume":"43","author":"Ng","year":"2023","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib26","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-020-67941-3","article-title":"Enhanced Notch3 signaling contributes to pulmonary emphysema in a Murine Model of Marfan syndrome","volume":"10","author":"Jespersen","year":"2020","journal-title":"Sci. Rep."},{"key":"10.1016\/j.jbc.2024.107445_bib27","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1038\/415168a","article-title":"Fibulin-5 is an elastin-binding protein essential for elastic fibre development in\u00a0vivo","volume":"415","author":"Yanagisawa","year":"2002","journal-title":"Nature"},{"key":"10.1016\/j.jbc.2024.107445_bib28","doi-asserted-by":"crossref","first-page":"3723","DOI":"10.1242\/dev.008599","article-title":"Long form of latent TGF-beta binding protein 1 (Ltbp1L) is essential for cardiac outflow tract septation and remodeling","volume":"134","author":"Todorovic","year":"2007","journal-title":"Development"},{"key":"10.1016\/j.jbc.2024.107445_bib29","doi-asserted-by":"crossref","first-page":"8016","DOI":"10.1074\/jbc.M511599200","article-title":"Fibrillins 1 and 2 Perform Partially overlapping functions during aortic development","volume":"281","author":"Carta","year":"2006","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib30","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1083\/jcb.200105046","article-title":"Regulation of limb patterning by extracellular microfibrils","volume":"154","author":"Arteaga-Solis","year":"2001","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib31","doi-asserted-by":"crossref","first-page":"3819","DOI":"10.1073\/pnas.96.7.3819","article-title":"Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1","volume":"96","author":"Pereira","year":"1999","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2024.107445_bib32","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1038\/modpathol.3801004","article-title":"Aberrant fibrillin-1 expression in early emphysematous human lung: a proposed predisposition for emphysema","volume":"21","author":"Robbesom","year":"2008","journal-title":"Mod. Pathol."},{"key":"10.1016\/j.jbc.2024.107445_bib33","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1513\/pats.200601-009AW","article-title":"The stumbling block in lung repair of emphysema: elastic fiber assembly","volume":"3","author":"Shifren","year":"2006","journal-title":"Proc. Am. Thorac. Soc."},{"key":"10.1016\/j.jbc.2024.107445_bib34","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1165\/ajrcmb.23.3.3906","article-title":"Impaired distal Airway development in mice lacking elastin","volume":"23","author":"Wendel","year":"2000","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib35","article-title":"Impact of Notch3 activation on aortic aneurysm development in marfan syndrome","volume":"2022","author":"Jespersen","year":"2022","journal-title":"J.\u00a0Immunol. Res."},{"key":"10.1016\/j.jbc.2024.107445_bib36","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1093\/hmg\/10.8.835","article-title":"Mutation of the gene encoding fibrillin-2 results in syndactyly in mice","volume":"10","author":"Chaudhry","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.jbc.2024.107445_bib37","series-title":"Chapter three - Lung Organogenesis","first-page":"73","article-title":"Current Topics in Developmental Biology","author":"Warburton","year":"2010"},{"key":"10.1016\/j.jbc.2024.107445_bib38","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/j.devcel.2018.01.008","article-title":"The strength of mechanical forces determines the differentiation of alveolar epithelial cells","volume":"44","author":"Li","year":"2018","journal-title":"Dev. Cell"},{"key":"10.1016\/j.jbc.2024.107445_bib39","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1016\/j.mod.2007.10.013","article-title":"Tracheal occlusion increases the rate of epithelial branching of embryonic mouse lung via the FGF10-FGFR2b-Sprouty2 pathway","volume":"125","author":"Unbekandt","year":"2008","journal-title":"Mech. Dev."},{"key":"10.1016\/j.jbc.2024.107445_bib40","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1161\/hq0701.092136","article-title":"Aortic wall Mechanics and Composition in a Transgenic mouse model of marfan syndrome","volume":"21","author":"Marque","year":"2001","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib41","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0152124","article-title":"Early Impairment of lung Mechanics in a murine model of marfan syndrome","volume":"11","author":"Uriarte","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.jbc.2024.107445_bib42","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.matbio.2019.06.006","article-title":"The role of fibrillin and microfibril binding proteins in elastin and elastic fibre assembly","volume":"84","author":"Godwin","year":"2019","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib43","doi-asserted-by":"crossref","first-page":"24400","DOI":"10.1074\/jbc.M003665200","article-title":"Interaction of tropoelastin with the amino-terminal domains of fibrillin-1 and fibrillin-2 suggests a role for the fibrillins in elastic fiber assembly","volume":"275","author":"Trask","year":"2000","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib44","doi-asserted-by":"crossref","first-page":"1628","DOI":"10.1016\/j.jvs.2012.10.007","article-title":"The fibrillin-1 hypomorphic mgR\/mgR murine model of Marfan syndrome shows severe elastolysis in all segments of the aorta","volume":"57","author":"Schwill","year":"2013","journal-title":"J.\u00a0Vasc. Surg."},{"key":"10.1016\/j.jbc.2024.107445_bib45","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1161\/01.RES.88.1.37","article-title":"Phenotypic alteration of vascular smooth muscle cells precede elastolysis in a mouse mode of marfan syndrome","volume":"88","author":"Bunton","year":"2001","journal-title":"Circ. Res."},{"key":"10.1016\/j.jbc.2024.107445_bib46","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1161\/01.ATV.17.4.665","article-title":"Differentiation of smooth muscle cells in human blood vessels as defined by smoothelin, a novel marker for the contractile phenotype","volume":"17","author":"van der Loop","year":"1997","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib47","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1016\/j.matbio.2022.04.004","article-title":"LTBP1 promotes fibrillin incorporation into the extracellular matrix","volume":"110","author":"Przyklenk","year":"2022","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib48","doi-asserted-by":"crossref","first-page":"3828","DOI":"10.1002\/jcp.24094","article-title":"Specificity of latent TGF-beta binding protein (LTBP) incorporation into matrix: role of fibrillins and fibronectin","volume":"227","author":"Zilberberg","year":"2012","journal-title":"J.\u00a0Cell Physiol."},{"key":"10.1016\/j.jbc.2024.107445_bib49","doi-asserted-by":"crossref","first-page":"18871","DOI":"10.1074\/jbc.M410762200","article-title":"Fibronectin regulates latent transforming growth factor-beta (TGF beta) by controlling matrix assembly of latent TGF beta-binding protein-1","volume":"280","author":"Dallas","year":"2005","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib50","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1091\/mbc.e08-08-0830","article-title":"Fibrillin assembly requires fibronectin","volume":"20","author":"Sabatier","year":"2009","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.jbc.2024.107445_bib51","doi-asserted-by":"crossref","first-page":"2696","DOI":"10.1242\/jcs.029819","article-title":"Fibrillin-1 microfibril deposition is dependent on fibronectin assembly","volume":"121","author":"Kinsey","year":"2008","journal-title":"J.\u00a0Cell Sci."},{"key":"10.1016\/j.jbc.2024.107445_bib52","doi-asserted-by":"crossref","first-page":"15","DOI":"10.3390\/proteomes9010015","article-title":"Proteome Discoverer-A Community Enhanced data processing suite for protein Informatics","volume":"9","author":"Orsburn","year":"2021","journal-title":"Proteomes"},{"key":"10.1016\/j.jbc.2024.107445_bib53","doi-asserted-by":"crossref","first-page":"5296","DOI":"10.1021\/pr200780j","article-title":"Two-dimensional target decoy strategy for shotgun proteomics","volume":"10","author":"Bern","year":"2011","journal-title":"J.\u00a0Proteome Res."},{"key":"10.1016\/j.jbc.2024.107445_bib54","doi-asserted-by":"crossref","first-page":"1892","DOI":"10.3390\/ijms23031892","article-title":"The multiple functions of fibrillin-1 microfibrils in Organismal Physiology","volume":"23","author":"Asano","year":"2022","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.jbc.2024.107445_bib55","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1038\/ng1116","article-title":"Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome","volume":"33","author":"Neptune","year":"2003","journal-title":"Nat. Genet."},{"key":"10.1016\/j.jbc.2024.107445_bib56","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1172\/JCI200420641","article-title":"Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome","volume":"114","author":"Judge","year":"2004","journal-title":"J.\u00a0Clin. Invest."},{"key":"10.1016\/j.jbc.2024.107445_bib57","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.71142","article-title":"Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development","volume":"11","author":"Mead","year":"2022","journal-title":"Elife"},{"key":"10.1016\/j.jbc.2024.107445_bib58","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1242\/dmm.017046","article-title":"Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia--a novel mouse model providing insights into geleophysic dysplasia","volume":"8","author":"Hubmacher","year":"2015","journal-title":"Dis. Model Mech."},{"key":"10.1016\/j.jbc.2024.107445_bib59","doi-asserted-by":"crossref","first-page":"1700","DOI":"10.1128\/MCB.26.5.1700-1709.2006","article-title":"Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice","volume":"26","author":"McLaughlin","year":"2006","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib60","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1038\/415171a","article-title":"Fibulin-5\/DANCE is essential for elastogenesis in\u00a0vivo","volume":"415","author":"Nakamura","year":"2002","journal-title":"Nature"},{"key":"10.1016\/j.jbc.2024.107445_bib61","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1016\/j.sbi.2018.12.005","article-title":"Protein O-fucosylation: structure and function","volume":"56","author":"Holdener","year":"2019","journal-title":"Curr. Opin. Struct. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib62","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/j.bbrc.2020.03.066","article-title":"Contribution of extracellular O-GlcNAc to the stability of folded epidermal growth factor-like domains and Notch1 trafficking","volume":"526","author":"Ogawa","year":"2020","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.jbc.2024.107445_bib63","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1002\/prot.1127","article-title":"Role of Ca2+ for the mechanical properties of fibrillin","volume":"45","author":"Eriksen","year":"2001","journal-title":"Proteins"},{"key":"10.1016\/j.jbc.2024.107445_bib64","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1177\/39.4.2005373","article-title":"Extraction of extendable beaded structures and their identification as fibrillin-containing extracellular matrix microfibrils","volume":"39","author":"Keene","year":"1991","journal-title":"J.\u00a0Histochem. Cytochem."},{"key":"10.1016\/j.jbc.2024.107445_bib65","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-020-73969-2","article-title":"Steered Molecular dynamic Simulations reveal marfan syndrome mutations Disrupt fibrillin-1 cbEGF domain Mechanosensitive Balcium binding","volume":"10","author":"Haller","year":"2020","journal-title":"Sci. Rep."},{"key":"10.1016\/j.jbc.2024.107445_bib66","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/S0167-4889(00)00085-9","article-title":"Fibrillin-1, a calcium binding protein of extracellular matrix","volume":"1498","author":"Handford","year":"2000","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.jbc.2024.107445_bib67","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1006\/jmbi.1997.1593","article-title":"Metal ion dependency of microfibrils supports a Rod-like conformation for fibrillin-1 calcium-binding epidermal growth factor-like domains","volume":"276","author":"Cardy","year":"1998","journal-title":"J.\u00a0Mol. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib68","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1126\/science.1261093","article-title":"Structural biology. Structural basis for Notch1 engagement of Delta-like 4","volume":"347","author":"Luca","year":"2015","journal-title":"Science"},{"key":"10.1016\/j.jbc.2024.107445_bib69","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1074\/jbc.272.2.1231","article-title":"Calcium stabilizes fibrillin-1 against proteolytic degradation","volume":"272","author":"Reinhardt","year":"1997","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2024.107445_bib70","doi-asserted-by":"crossref","first-page":"1320","DOI":"10.1126\/science.aaf9739","article-title":"Notch-Jagged complex structure implicates a catch bond in tuning ligand sensitivity","volume":"355","author":"Luca","year":"2017","journal-title":"Science"},{"key":"10.1016\/j.jbc.2024.107445_bib71","doi-asserted-by":"crossref","first-page":"11922","DOI":"10.1073\/pnas.0601609103","article-title":"Nanostructure of fibrillin-1 reveals compact conformation of EGF arrays and mechanism for extensibility","volume":"103","author":"Baldock","year":"2006","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2024.107445_bib72","series-title":"Microfibril-Associated Glycoprotein-1 (MAGP-1) and Other Non-fibrillin Macromolecules Which May Possess a Functional Association with the 10 nm Microfibrils. Madame Curie Bioscience Database","author":"Gibson","year":"2000"},{"key":"10.1016\/j.jbc.2024.107445_bib73","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.matbio.2015.05.003","article-title":"The microfibril-associated glycoproteins (MAGPs) and the microfibrillar niche","volume":"47","author":"Mecham","year":"2015","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib74","doi-asserted-by":"crossref","DOI":"10.1016\/j.arr.2021.101255","article-title":"Elastic fibers during aging and disease","volume":"66","author":"Heinz","year":"2021","journal-title":"Ageing Res. Rev."},{"key":"10.1016\/j.jbc.2024.107445_bib75","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1161\/CIRCRESAHA.109.207852","article-title":"Fibulin-4 deficiency results in ascending aortic aneurysms: a potential link between abnormal smooth muscle cell phenotype and aneurysm progression","volume":"106","author":"Huang","year":"2010","journal-title":"Circ. Res."},{"key":"10.1016\/j.jbc.2024.107445_bib76","doi-asserted-by":"crossref","first-page":"12601","DOI":"10.1021\/bi8005384","article-title":"Fibrillins, fibulins, and matrix-associated glycoprotein modulate the kinetics and morphology of in\u00a0vitro self-assembly of a recombinant elastin-like polypeptide","volume":"47","author":"Cirulis","year":"2008","journal-title":"Biochemistry"},{"key":"10.1016\/j.jbc.2024.107445_bib77","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1083\/jcb.200611026","article-title":"Fibulin-5\/DANCE has an elastogenic organizer activity that is abrogated by proteolytic cleavage in\u00a0vivo","volume":"176","author":"Hirai","year":"2007","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib78","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1002\/dvdy.271","article-title":"A\u00a0cell-centric view of lung alveologenesis","volume":"250","author":"Vila Ellis","year":"2021","journal-title":"Dev. Dyn."},{"key":"10.1016\/j.jbc.2024.107445_bib79","doi-asserted-by":"crossref","DOI":"10.3390\/ijms222212107","article-title":"Alveologenesis: what Governs secondary septa formation","volume":"22","author":"Rippa","year":"2021","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.jbc.2024.107445_bib80","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1016\/j.ydbio.2015.11.017","article-title":"A\u00a0three-dimensional study of alveologenesis in mouse lung","volume":"409","author":"Branchfield","year":"2016","journal-title":"Dev. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib81","doi-asserted-by":"crossref","first-page":"4087","DOI":"10.3390\/ijms23084087","article-title":"Elastic fibre proteins in elastogenesis and Wound Healing","volume":"23","author":"Zhang","year":"2022","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.jbc.2024.107445_bib82","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1038\/30522","article-title":"Elastin is an essential determinant of arterial morphogenesis","volume":"393","author":"Li","year":"1998","journal-title":"Nature"},{"key":"10.1016\/j.jbc.2024.107445_bib83","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1038\/s41594-023-00950-8","article-title":"Fibrillin microfibril structure identifies long-range effects of inherited pathogenic mutations affecting a key regulatory latent TGFbeta-binding site","volume":"30","author":"Godwin","year":"2023","journal-title":"Nat. Struct. Mol. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib84","doi-asserted-by":"crossref","first-page":"7561","DOI":"10.3390\/ijms24087561","article-title":"Nanoscale structural comparison of fibrillin-1 microfibrils isolated from marfan and non-marfan syndrome human aorta","volume":"24","author":"Sulea","year":"2023","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.jbc.2024.107445_bib85","doi-asserted-by":"crossref","first-page":"D325","DOI":"10.1093\/nar\/gkaa1113","article-title":"The Gene Ontology resource: enriching a GOld mine","volume":"49","author":"Gene Ontology","year":"2021","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.jbc.2024.107445_bib86","doi-asserted-by":"crossref","first-page":"D419","DOI":"10.1093\/nar\/gky1038","article-title":"PANTHER version 14: more genomes, a new PANTHER GO-slim and improvements in enrichment analysis tools","volume":"47","author":"Mi","year":"2019","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.jbc.2024.107445_bib87","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1038\/75556","article-title":"Gene ontology: tool for the unification of biology. The Gene Ontology Consortium","volume":"25","author":"Ashburner","year":"2000","journal-title":"Nat. Genet."},{"key":"10.1016\/j.jbc.2024.107445_bib88","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.matbio.2022.02.002","article-title":"O-fucosylation of thrombospondin type 1 repeats is essential for ECM remodeling and signaling during bone development","volume":"107","author":"Neupane","year":"2022","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib89","doi-asserted-by":"crossref","DOI":"10.3389\/fcell.2021.697243","article-title":"Signaling modulation by miRNA-221-3p during Tooth morphogenesis in mice","volume":"9","author":"Aryal","year":"2021","journal-title":"Front. Cell Dev. Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib90","doi-asserted-by":"crossref","first-page":"1499","DOI":"10.1002\/jcp.22479","article-title":"Control of lung development by latent TGF-beta binding proteins","volume":"226","author":"Dabovic","year":"2011","journal-title":"J.\u00a0Cell Physiol."},{"key":"10.1016\/j.jbc.2024.107445_bib91","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.matbio.2023.02.003","article-title":"A\u00a0new mouse model of elastin haploinsufficiency highlights the importance of elastin to vascular development and blood pressure regulation","volume":"117","author":"Brengle","year":"2023","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2024.107445_bib92","doi-asserted-by":"crossref","first-page":"175","DOI":"10.3758\/BF03193146","article-title":"G\u2217Power 3: a flexible statistical power analysis program for the social, behavioral, and biomedical sciences","volume":"39","author":"Faul","year":"2007","journal-title":"Behav. Res. Methods"},{"key":"10.1016\/j.jbc.2024.107445_bib93","doi-asserted-by":"crossref","first-page":"D442","DOI":"10.1093\/nar\/gky1106","article-title":"The PRIDE database and related tools and resources in 2019: improving support for quantification data","volume":"47","author":"Perez-Riverol","year":"2019","journal-title":"Nucleic Acids Res."}],"container-title":["Journal of Biological Chemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S002192582401946X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S002192582401946X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,10,6]],"date-time":"2024-10-06T21:40:20Z","timestamp":1728250820000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S002192582401946X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7]]},"references-count":93,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2024,7]]}},"alternative-id":["S002192582401946X"],"URL":"https:\/\/doi.org\/10.1016\/j.jbc.2024.107445","relation":{},"ISSN":["0021-9258"],"issn-type":[{"value":"0021-9258","type":"print"}],"subject":[],"published":{"date-parts":[[2024,7]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Poglut2\/3 double knockout in mice results in neonatal lethality with reduced levels of fibrillin in lung tissues","name":"articletitle","label":"Article Title"},{"value":"Journal of Biological Chemistry","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jbc.2024.107445","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Authors. Published by Elsevier Inc on behalf of American Society for Biochemistry and Molecular Biology.","name":"copyright","label":"Copyright"}],"article-number":"107445"}}