Isoform switching of type IV collagen is developmentally arrested in X-linked Alport syndrome leading to increased susceptibility of renal basement membranes to endoproteolysis.

Normal glomerular capillaries filter plasma through a basement membrane (GBM) rich in alpha3(IV), alpha4(IV), and alpha5(IV) chains of type IV collagen. We now show that these latter isoforms are absent biochemically from the glomeruli in patients with X-linked Alport syndrome (XAS). Their GBM inste...

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Bibliographic Details
Published in:J Clin Invest
Main Authors: Kalluri, R, Shield, C F, Todd, P, Hudson, B G, Neilson, E G
Format: Artigo
Language:Inglês
Published: American Society for Clinical Investigation 1997
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Research Article
Online Access:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC508088/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/9153291/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI119431
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