Article
Collagen IVα345 dysfunction in glomerular basement membrane diseases. I. Discovery of a COL4A3 variant in familial Goodpasture's and Alport diseases.
The Journal of biological chemistry - 1 Jan 2000
Pokidysheva Elena N, Seeger Harald, Pedchenko Vadim, Chetyrkin Sergei, Bergmann Carsten, Abrahamson Dale, Cui Zhao Wei, Delpire Eric, Fervenza Fernando C, Fidler Aaron L, Fogo Agnes B, Gaspert Ariana, Grohmann Maik, Gross Oliver, Haddad George, Harris Raymond C, Kashtan Clifford, Kitching A Richard, Lorenzen Johan M, McAdoo Stephen, Pusey Charles D, Segelmark Marten, Simmons Alicia, Voziyan Paul A, Wagner Timo, Wüthrich Rudolf P, Zhao Ming-Hui, Boudko Sergei P, Kistler Andreas D, Hudson Billy G
Abstract excerpt
Diseases of the glomerular basement membrane (GBM), such as Goodpasture's disease (GP) and Alport syndrome (AS), are a major cause of chronic kidney failure and an unmet medical need. Collagen IVα345 is an important architectural element of the GBM that was discovered in previous research on GP and AS. How this collagen enables GBM to function as a permselective filter and how structural defects cause renal...
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