Collagen IV diseases: A focus on the glomerular basement membrane in Alport syndrome

Dominic Cosgrove, Shiguang Liu

Research output: Contribution to journalReview article

28 Scopus citations

Abstract

Alport syndrome is the result of mutations in any of three type IV collagen genes, COL4A3, COL4A4, or COL4A5. Because the three collagen chains form heterotrimers, there is an absence of all three proteins in the basement membranes where they are expressed. In the glomerulus, the mature glomerular basement membrane type IV collagen network, normally comprised of two separate networks, α3(IV)/α4(IV)/α5(IV) and α1(IV)/α2(IV), is comprised entirely of collagen α1(IV)/α2. This review addresses the current state of our knowledge regarding the consequence of this change in basement membrane composition, including both the direct, via collagen receptor binding, and indirect, regarding influences on glomerular biomechanics. The state of our current understanding regarding mechanisms of glomerular disease initiation and progression will be examined, as will the current state of the art regarding emergent therapeutic approaches to slow or arrest glomerular disease in Alport patients.

Original languageEnglish (US)
Pages (from-to)45-54
Number of pages10
JournalMatrix Biology
Volume57-58
DOIs
StatePublished - Jan 1 2017

ASJC Scopus subject areas

  • Molecular Biology

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