Article
Collagen IVα345 dysfunction in glomerular basement membrane diseases. II. Crystal structure of the α345 hexamer.
The Journal of biological chemistry - 1 Jan 2000
Boudko Sergei P, Bauer Ryan, Chetyrkin Sergei V, Ivanov Sergey, Smith Jarrod, Voziyan Paul A, Hudson Billy G
Abstract excerpt
Our recent work identified a genetic variant of the α345 hexamer of the collagen IV scaffold that is present in patients with glomerular basement membrane diseases, Goodpasture's disease (GP) and Alport syndrome (AS), and phenocopies of AS in knock-in mice. To understand the context of this "Zurich" variant, an 8-amino acid appendage, we developed a construct of the WT α345 hexamer using the single-chain NC1...
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