{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T13:32:09Z","timestamp":1791898329917,"version":"4.3.4"},"reference-count":75,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"7","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["CJASN"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:p>\n                    The glomerular basement membrane is a vital component of the filtration barrier of the kidney and is primarily composed of a highly structured matrix of type IV collagen. Specific isoforms of type IV collagen, the\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    3(IV),\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    4(IV), and\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    5(IV) isoforms, assemble into trimers that are required for normal glomerular basement membrane function. Disruption or alteration in these isoforms leads to breakdown of the glomerular basement membrane structure and function and can lead to progressive CKD known as Alport syndrome. However, there is wide variability in phenotype among patients with mutations affecting type IV collagen that depends on a complex interplay of sex, genotype, and X-chromosome inactivation. This article reviews the genetic basis of collagen disorders of the kidney as well as potential treatments for these conditions, including direct alteration of the DNA, RNA therapies, and manipulation of collagen proteins.\n                  <\/jats:p>","DOI":"10.2215\/cjn.19171220","type":"journal-article","created":{"date-parts":[[2021,4,13]],"date-time":"2021-04-13T12:36:41Z","timestamp":1618317401000},"page":"1101-1109","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":71,"title":["Genetic Basis of Type IV Collagen Disorders of the Kidney"],"prefix":"10.2215","volume":"16","clinical-trial-number":[{"clinical-trial-number":"nct02855268","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03019185","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03728322","registry":"10.18810\/clinical-trials-gov"}],"author":[{"given":"Catherine","family":"Quinlan","sequence":"first","affiliation":[{"name":"Department of Nephrology, Royal Children\u2019s Hospital, Melbourne, Victoria, Australia"},{"name":"Department of Kidney Regeneration, Murdoch Children\u2019s Research Institute, Melbourne, Victoria, Australia"},{"name":"Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2494-3970","authenticated-orcid":false,"given":"Michelle N.","family":"Rheault","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology, Department of Pediatrics, University of Minnesota Masonic Children\u2019s Hospital, Minneapolis, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2021,7]]},"reference":[{"key":"B1-20241015","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":"B2-20241015","doi-asserted-by":"crossref","first-page":"8767","DOI":"10.1074\/jbc.273.15.8767","article-title":"Glomerular basement membrane. 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