{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,7]],"date-time":"2026-03-07T11:01:41Z","timestamp":1772881301356,"version":"3.50.1"},"reference-count":65,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:00:00Z","timestamp":1609459200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:00:00Z","timestamp":1609459200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,3,24]],"date-time":"2021-03-24T00:00:00Z","timestamp":1616544000000},"content-version":"vor","delay-in-days":82,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","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":[[2021,1]]},"DOI":"10.1016\/j.jbc.2021.100590","type":"journal-article","created":{"date-parts":[[2021,3,26]],"date-time":"2021-03-26T17:57:08Z","timestamp":1616781428000},"page":"100590","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":30,"special_numbering":"C","title":["Collagen IV\u03b1345 dysfunction in glomerular basement membrane diseases. I. Discovery of a COL4A3 variant in familial Goodpasture\u2019s and Alport diseases"],"prefix":"10.1016","volume":"296","author":[{"given":"Elena N.","family":"Pokidysheva","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Harald","family":"Seeger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vadim","family":"Pedchenko","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sergei","family":"Chetyrkin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carsten","family":"Bergmann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dale","family":"Abrahamson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhao Wei","family":"Cui","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric","family":"Delpire","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9952-209X","authenticated-orcid":false,"given":"Fernando C.","family":"Fervenza","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2519-8864","authenticated-orcid":false,"given":"Aaron L.","family":"Fidler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Agnes B.","family":"Fogo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ariana","family":"Gaspert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maik","family":"Grohmann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8390-8852","authenticated-orcid":false,"given":"Oliver","family":"Gross","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"George","family":"Haddad","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raymond C.","family":"Harris","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clifford","family":"Kashtan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2713-2391","authenticated-orcid":false,"given":"A. Richard","family":"Kitching","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johan M.","family":"Lorenzen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen","family":"McAdoo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charles D.","family":"Pusey","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1221-0772","authenticated-orcid":false,"given":"Marten","family":"Segelmark","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alicia","family":"Simmons","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul A.","family":"Voziyan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Timo","family":"Wagner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rudolf P.","family":"W\u00fcthrich","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ming-Hui","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3856-4859","authenticated-orcid":false,"given":"Sergei P.","family":"Boudko","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas D.","family":"Kistler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5420-4100","authenticated-orcid":false,"given":"Billy G.","family":"Hudson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.jbc.2021.100590_bib1","doi-asserted-by":"crossref","first-page":"1888","DOI":"10.1016\/S0140-6736(17)30788-2","article-title":"Global kidney health 2017 and beyond: A roadmap for closing gaps in care, research, and policy","volume":"390","author":"Levin","year":"2017","journal-title":"Lancet"},{"key":"10.1016\/j.jbc.2021.100590_bib2","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1159\/000489897","article-title":"Disparities in chronic kidney disease prevalence among males and females in 195 countries: Analysis of the global burden of disease 2016 study","volume":"139","author":"Bikbov","year":"2018","journal-title":"Nephron"},{"key":"10.1016\/j.jbc.2021.100590_bib3","doi-asserted-by":"crossref","first-page":"26033","DOI":"10.1016\/S0021-9258(19)74270-7","article-title":"Type IV collagen: Structure, gene organization, and role in human diseases. Molecular basis of goodpasture and Alport syndromes and diffuse leiomyomatosis","volume":"268","author":"Hudson","year":"1993","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib4","doi-asserted-by":"crossref","first-page":"2543","DOI":"10.1056\/NEJMra022296","article-title":"Alport's syndrome, Goodpasture's syndrome, and type IV collagen","volume":"348","author":"Hudson","year":"2003","journal-title":"N.\u00a0Engl. J. Med."},{"key":"10.1016\/j.jbc.2021.100590_bib5","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1038\/s41581-020-0329-y","article-title":"Complexities of the glomerular basement membrane","volume":"17","author":"Naylor","year":"2021","journal-title":"Nat. Rev. Nephrol."},{"key":"10.1016\/j.jbc.2021.100590_bib6","doi-asserted-by":"crossref","first-page":"3103","DOI":"10.1021\/bi00849a012","article-title":"Isolation and characterization of the collagen from glomerular basement membrane","volume":"7","author":"Kefalides","year":"1968","journal-title":"Biochemistry"},{"key":"10.1016\/j.jbc.2021.100590_bib7","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/B978-0-12-363706-2.50008-8","article-title":"Structure and biosynthesis of basement membranes","volume":"6","author":"Kefalides","year":"1973","journal-title":"Int. Rev. Connect. Tissue Res."},{"key":"10.1016\/j.jbc.2021.100590_bib8","doi-asserted-by":"crossref","first-page":"1915","DOI":"10.1016\/S0021-9258(18)96088-6","article-title":"Studies on the renal glomerular basement membrane. Preparation and chemical composition","volume":"242","author":"Spiro","year":"1967","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib9","doi-asserted-by":"crossref","first-page":"596","DOI":"10.1126\/science.168.3931.596","article-title":"Human glomerular basement membrane: Chemical alteration in diabetes mellitus","volume":"168","author":"Beisswenger","year":"1970","journal-title":"Science"},{"key":"10.1016\/j.jbc.2021.100590_bib10","doi-asserted-by":"crossref","first-page":"4229","DOI":"10.1016\/S0021-9258(19)45065-5","article-title":"Studies on the native and reduced alkylated renal glomerular basement membrane. Solubility, subunit size, and reaction with cyanogen bromide","volume":"247","author":"Hudson","year":"1972","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib11","doi-asserted-by":"crossref","first-page":"1337","DOI":"10.1056\/NEJM197306212882506","article-title":"Biochemistry of the renal glomerular basement membrane and its alterations in diabetes mellitus","volume":"288","author":"Spiro","year":"1973","journal-title":"N.\u00a0Engl. J. Med."},{"key":"10.1016\/j.jbc.2021.100590_bib12","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.1038\/ki.1997.455","article-title":"Nonenzymatic glycation of type IV collagen and matrix metalloproteinase susceptibility","volume":"52","author":"Mott","year":"1997","journal-title":"Kidney Int."},{"key":"10.1016\/j.jbc.2021.100590_bib13","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1111\/j.1432-1033.1981.tb05690.x","article-title":"A\u00a0network model for the organization of type IV collagen molecules in basement membranes","volume":"120","author":"Timpl","year":"1981","journal-title":"Eur. J. Biochem."},{"key":"10.1016\/j.jbc.2021.100590_bib14","doi-asserted-by":"crossref","first-page":"2559","DOI":"10.1083\/jcb.105.6.2559","article-title":"Basement membrane structure in situ: Evidence for lateral associations in the type IV collagen network","volume":"105","author":"Yurchenco","year":"1987","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.jbc.2021.100590_bib15","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1038\/ki.1982.72","article-title":"Absence of nephritogenic GBM antigen(s) in some patients with hereditary nephritis","volume":"21","author":"McCoy","year":"1982","journal-title":"Kidney Int."},{"key":"10.1016\/j.jbc.2021.100590_bib16","doi-asserted-by":"crossref","first-page":"7874","DOI":"10.1016\/S0021-9258(18)47648-X","article-title":"Localization of the Goodpasture epitope to a novel chain of basement membrane collagen","volume":"262","author":"Butkowski","year":"1987","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib17","doi-asserted-by":"crossref","first-page":"13374","DOI":"10.1016\/S0021-9258(18)37714-7","article-title":"Identification of the Goodpasture antigen as the alpha 3(IV) chain of collagen IV","volume":"263","author":"Saus","year":"1988","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib18","doi-asserted-by":"crossref","first-page":"1224","DOI":"10.1126\/science.2349482","article-title":"Identification of mutations in the COL4A5 collagen gene in Alport syndrome","volume":"248","author":"Barker","year":"1990","journal-title":"Science"},{"key":"10.1016\/j.jbc.2021.100590_bib19","doi-asserted-by":"crossref","first-page":"5466","DOI":"10.1016\/S0021-9258(19)39384-6","article-title":"Glomerular basement membrane. Identification of a fourth chain, alpha 4, of type IV collagen","volume":"265","author":"Gunwar","year":"1990","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib20","doi-asserted-by":"crossref","first-page":"1606","DOI":"10.1073\/pnas.87.4.1606","article-title":"Identification of a distinct type IV collagen alpha chain with restricted kidney distribution and assignment of its gene to the locus of X chromosome-linked Alport syndrome","volume":"87","author":"Hostikka","year":"1990","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2021.100590_bib21","first-page":"1024","article-title":"Molecular cloning of alpha 5(IV) collagen and assignment of the gene to the region of the X chromosome containing the Alport syndrome locus","volume":"46","author":"Myers","year":"1990","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.jbc.2021.100590_bib22","doi-asserted-by":"crossref","first-page":"38117","DOI":"10.1074\/jbc.R600025200","article-title":"Molecular recognition in the assembly of collagens: Terminal noncollagenous domains are key recognition modules in the formation of triple helical protomers","volume":"281","author":"Khoshnoodi","year":"2006","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib23","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1002\/jemt.20564","article-title":"Mammalian collagen IV","volume":"71","author":"Khoshnoodi","year":"2008","journal-title":"Microsc. Res. Tech."},{"key":"10.1016\/j.jbc.2021.100590_bib24","doi-asserted-by":"crossref","first-page":"1219","DOI":"10.1083\/jcb.130.5.1219","article-title":"Differential expression of two basement membrane collagen genes, COL4A6 and COL4A5, demonstrated by immunofluorescence staining using peptide-specific monoclonal antibodies","volume":"130","author":"Ninomiya","year":"1995","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.jbc.2021.100590_bib25","doi-asserted-by":"crossref","first-page":"2514","DOI":"10.1097\/01.ASN.0000141462.00630.76","article-title":"The molecular basis of Goodpasture and Alport syndromes: Beacons for the discovery of the collagen IV family","volume":"15","author":"Hudson","year":"2004","journal-title":"J.\u00a0Am. Soc. Nephrol."},{"key":"10.1016\/j.jbc.2021.100590_bib26","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.24176","article-title":"Collagen IV and basement membrane at the evolutionary dawn of metazoan tissues","volume":"6","author":"Fidler","year":"2017","journal-title":"Elife"},{"key":"10.1016\/j.jbc.2021.100590_bib27","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1056\/NEJMoa0910500","article-title":"Molecular architecture of the Goodpasture autoantigen in anti-GBM nephritis","volume":"363","author":"Pedchenko","year":"2010","journal-title":"N.\u00a0Engl. J. Med."},{"key":"10.1016\/j.jbc.2021.100590_bib28","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1165\/ajrcmb\/5.2.107","article-title":"Alveolar basement membrane: Molecular properties of the noncollagenous domain (hexamer) of collagen IV and its reactivity with Goodpasture autoantibodies","volume":"5","author":"Gunwar","year":"1991","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"10.1016\/j.jbc.2021.100590_bib29","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1172\/JCI115625","article-title":"Molecular cloning of the human Goodpasture antigen demonstrates it to be the alpha 3 chain of type IV collagen","volume":"89","author":"Turner","year":"1992","journal-title":"J.\u00a0Clin. Invest."},{"key":"10.1016\/j.jbc.2021.100590_bib30","first-page":"545","article-title":"Sequence and localization of a partial cDNA encoding the human alpha 3 chain of type IV collagen","volume":"49","author":"Morrison","year":"1991","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.jbc.2021.100590_bib31","doi-asserted-by":"crossref","first-page":"11267","DOI":"10.1074\/jbc.274.16.11267","article-title":"The Goodpasture autoantigen. Mapping the major conformational epitope(s) of alpha3(IV) collagen to residues 17-31 and 127-141 of the NC1 domain","volume":"274","author":"Netzer","year":"1999","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib32","doi-asserted-by":"crossref","first-page":"25862","DOI":"10.1074\/jbc.274.36.25862","article-title":"Goodpasture disease. Characterization of a single conformational epitope as the target of pathogenic autoantibodies","volume":"274","author":"Hellmark","year":"1999","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib33","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jaut.2016.04.001","article-title":"Antibodies to alpha5 chain of collagen IV are pathogenic in Goodpasture's disease","volume":"70","author":"Cui","year":"2016","journal-title":"J.\u00a0Autoimmun."},{"key":"10.1016\/j.jbc.2021.100590_bib34","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1016\/j.matbio.2018.05.004","article-title":"Goodpasture's autoimmune disease - a collagen IV disorder","volume":"71-72","author":"Pedchenko","year":"2018","journal-title":"Matrix Biol."},{"key":"10.1016\/j.jbc.2021.100590_bib35","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.2215\/CJN.01380217","article-title":"Anti-glomerular basement membrane disease","volume":"12","author":"McAdoo","year":"2017","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.1016\/j.jbc.2021.100590_bib36","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1056\/NEJMoa1806891","article-title":"Diagnostic utility of exome sequencing for kidney disease","volume":"380","author":"Groopman","year":"2019","journal-title":"N.\u00a0Engl. J. Med."},{"key":"10.1016\/j.jbc.2021.100590_bib37","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1016\/j.kint.2017.12.018","article-title":"Alport syndrome: A unified classification of genetic disorders of collagen IV alpha345: A position paper of the Alport Syndrome Classification Working Group","volume":"93","author":"Kashtan","year":"2018","journal-title":"Kidney Int."},{"key":"10.1016\/j.jbc.2021.100590_bib38","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1681\/ASN.2012020148","article-title":"Expert guidelines for the management of Alport syndrome and thin basement membrane nephropathy","volume":"24","author":"Savige","year":"2013","journal-title":"J.\u00a0Am. Soc. Nephrol."},{"key":"10.1016\/j.jbc.2021.100590_bib39","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0161802","article-title":"X-linked and autosomal recessive Alport syndrome: Pathogenic variant features and further genotype-phenotype correlations","volume":"11","author":"Savige","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.jbc.2021.100590_bib40","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1046\/j.1523-1755.1999.00601.x","article-title":"Making the diagnosis of Alport's syndrome","volume":"56","author":"Pirson","year":"1999","journal-title":"Kidney Int."},{"key":"10.1016\/j.jbc.2021.100590_bib41","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.1016\/j.kint.2019.12.015","article-title":"A\u00a0multicenter, randomized, placebo-controlled, double-blind phase 3 trial with open-arm comparison indicates safety and efficacy of nephroprotective therapy with ramipril in children with Alport's syndrome","volume":"97","author":"Gross","year":"2020","journal-title":"Kidney Int."},{"key":"10.1016\/j.jbc.2021.100590_bib42","series-title":"Journal of the American Society of Nephrology: JASN","article-title":"Familial Goodpasture\u2019s disease associated with a deletion in COL4A3: A potential clue to etiology","author":"Seeger","year":"2018"},{"key":"10.1016\/j.jbc.2021.100590_bib43","doi-asserted-by":"crossref","first-page":"277","DOI":"10.5414\/CN109206","article-title":"Familial antiglomerular basement membrane disease in zero human leukocyte antigen mismatch siblings","volume":"88","author":"Angioi","year":"2017","journal-title":"Clin. Nephrol."},{"key":"10.1016\/j.jbc.2021.100590_bib44","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1038\/nature22329","article-title":"Dominant protection from HLA-linked autoimmunity by antigen-specific regulatory T cells","volume":"545","author":"Ooi","year":"2017","journal-title":"Nature"},{"key":"10.1016\/j.jbc.2021.100590_bib45","doi-asserted-by":"crossref","first-page":"1638","DOI":"10.1046\/j.1523-1755.1999.00720.x","article-title":"The HLA complex in Goodpasture's disease: A model for analyzing susceptibility to autoimmunity","volume":"56","author":"Phelps","year":"1999","journal-title":"Kidney Int."},{"key":"10.1016\/j.jbc.2021.100590_bib46","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1111\/imm.12736","article-title":"The susceptible HLA class II alleles and their presenting epitope(s) in Goodpasture's disease","volume":"151","author":"Xie","year":"2017","journal-title":"Immunology"},{"key":"10.1016\/j.jbc.2021.100590_bib47","doi-asserted-by":"crossref","first-page":"2981","DOI":"10.1101\/gad.10.23.2981","article-title":"Collagen COL4A3 knockout: A mouse model for autosomal Alport syndrome","volume":"10","author":"Cosgrove","year":"1996","journal-title":"Genes Dev."},{"key":"10.1016\/j.jbc.2021.100590_bib48","doi-asserted-by":"crossref","first-page":"1403","DOI":"10.1083\/jcb.135.5.1403","article-title":"Molecular and functional defects in kidneys of mice lacking collagen alpha 3(IV): Implications for Alport syndrome","volume":"135","author":"Miner","year":"1996","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.jbc.2021.100590_bib49","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbc.2021.100591","article-title":"Collagen IV\u03b1345 dysfunction in glomerular basement membrane diseases. II. Crystal structure of the \u03b1345 hexamer","volume":"296","author":"Boudko","year":"2021","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib50","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbc.2021.100592","article-title":"Collagen IV\u03b1345 dysfunction in glomerular basement membrane diseases. III. A functional framework for \u03b1345 hexamer assembly","volume":"296","author":"Pedchenko","year":"2021","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib51","first-page":"375","article-title":"Applications of reactive cysteine profiling","volume":"420","author":"Backus","year":"2019","journal-title":"Curr. Top. Microbiol. Immunol."},{"key":"10.1016\/j.jbc.2021.100590_bib52","doi-asserted-by":"crossref","first-page":"4633","DOI":"10.1021\/cr300163e","article-title":"Cysteine-mediated redox signaling: Chemistry, biology, and tools for discovery","volume":"113","author":"Paulsen","year":"2013","journal-title":"Chem. Rev."},{"key":"10.1016\/j.jbc.2021.100590_bib53","doi-asserted-by":"crossref","first-page":"1426","DOI":"10.1681\/ASN.2017060690","article-title":"Laminin-521 protein therapy for glomerular basement membrane and podocyte abnormalities in a model of Pierson syndrome","volume":"29","author":"Lin","year":"2018","journal-title":"J.\u00a0Am. Soc. Nephrol."},{"key":"10.1016\/j.jbc.2021.100590_bib54","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1046\/j.1523-1755.1998.00795.x","article-title":"Induction of anti-GBM nepritis in rats by recombinant \u03b13(IV)NC1 and \u03b14(IV)NC1 of type IV collagen","volume":"53","author":"Sado","year":"1998","journal-title":"Kidney Int."},{"key":"10.1016\/j.jbc.2021.100590_bib55","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/bs.mcb.2017.08.010","article-title":"Basement membrane collagen IV: Isolation of functional domains","volume":"143","author":"Boudko","year":"2018","journal-title":"Methods Cell Biol."},{"key":"10.1016\/j.jbc.2021.100590_bib56","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1080\/019131290882286","article-title":"Variability of interalveolar septal remodeling after bleomycin treatment in mice","volume":"29","author":"Polosukhin","year":"2005","journal-title":"Ultrastruct. Pathol."},{"key":"10.1016\/j.jbc.2021.100590_bib57","doi-asserted-by":"crossref","first-page":"1947","DOI":"10.1046\/j.1523-1755.2002.00379.x","article-title":"COL4A3\/COL4A4 mutations: From familial hematuria to autosomal-dominant or recessive Alport syndrome","volume":"61","author":"Longo","year":"2002","journal-title":"Kidney Int."},{"key":"10.1016\/j.jbc.2021.100590_bib58","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/S1672-0229(07)60009-6","article-title":"A\u00a0brief review of short tandem repeat mutation","volume":"5","author":"Fan","year":"2007","journal-title":"Genomics Proteomics Bioinformatics"},{"key":"10.1016\/j.jbc.2021.100590_bib59","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1016\/j.ekir.2017.04.011","article-title":"Natural history and genotype-phenotype correlation in female X-linked Alport syndrome","volume":"2","author":"Yamamura","year":"2017","journal-title":"Kidney Int. Rep."},{"key":"10.1016\/j.jbc.2021.100590_bib60","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1681\/ASN.2007070793","article-title":"Human podocytes adhere to the KRGDS motif of the alpha3alpha4alpha5 collagen IV network","volume":"19","author":"Borza","year":"2008","journal-title":"J.\u00a0Am. Soc. Nephrol."},{"key":"10.1016\/j.jbc.2021.100590_bib61","doi-asserted-by":"crossref","first-page":"12642","DOI":"10.1074\/jbc.274.18.12642","article-title":"Characterization of a novel type of serine\/threonine kinase that specifically phosphorylates the human goodpasture antigen","volume":"274","author":"Raya","year":"1999","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.jbc.2021.100590_bib62","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1016\/j.semnephrol.2012.06.005","article-title":"Role of the podocyte (and glomerular endothelium) in building the GBM","volume":"32","author":"Abrahamson","year":"2012","journal-title":"Semin. Nephrol."},{"key":"10.1016\/j.jbc.2021.100590_bib63","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1083\/jcb.201510065","article-title":"Extracellular chloride signals collagen IV network assembly during basement membrane formation","volume":"213","author":"Cummings","year":"2016","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.jbc.2021.100590_bib64","doi-asserted-by":"crossref","first-page":"2151","DOI":"10.1002\/pro.3283","article-title":"Building collagen IV smart scaffolds on the outside of cells","volume":"26","author":"Brown","year":"2017","journal-title":"Protein Sci."},{"key":"10.1016\/j.jbc.2021.100590_bib65","doi-asserted-by":"crossref","first-page":"10691","DOI":"10.1021\/acs.chemrev.8b00753","article-title":"Weak chemical interactions that drive protein evolution: Crowding, sticking, and quinary structure in folding and function","volume":"119","author":"Guin","year":"2019","journal-title":"Chem. Rev."}],"container-title":["Journal of Biological Chemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925821003707?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925821003707?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,18]],"date-time":"2025-09-18T23:51:40Z","timestamp":1758239500000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0021925821003707"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1]]},"references-count":65,"alternative-id":["S0021925821003707"],"URL":"https:\/\/doi.org\/10.1016\/j.jbc.2021.100590","relation":{},"ISSN":["0021-9258"],"issn-type":[{"value":"0021-9258","type":"print"}],"subject":[],"published":{"date-parts":[[2021,1]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Collagen IV\u03b1345 dysfunction in glomerular basement membrane diseases. I. Discovery of a COL4A3 variant in familial Goodpasture\u2019s and Alport diseases","name":"articletitle","label":"Article Title"},{"value":"Journal of Biological Chemistry","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jbc.2021.100590","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"https:\/\/doi.org\/10.1016\/j.jbc.2021.100591","name":"associatedlink","label":"CrossRef DOI link to the associated document"},{"value":"https:\/\/doi.org\/10.1016\/j.jbc.2021.100592","name":"associatedlink","label":"CrossRef DOI link to the associated document"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 The Authors. Published by Elsevier Inc on behalf of American Society for Biochemistry and Molecular Biology.","name":"copyright","label":"Copyright"}],"article-number":"100590"}}