Collagen IV(α345) dysfunction in glomerular basement membrane diseases. III. A functional framework for α345 hexamer assembly

We identified a genetic variant, an 8-residue appendage, of the α345 hexamer of collagen IV present in patients with glomerular basement membrane diseases, Goodpasture’s disease and Alport syndrome, and determined the long-awaited crystal structure of the hexamer. We sought to elucidate how variants...

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Dettagli Bibliografici
Pubblicato in:J Biol Chem
Autori principali: Pedchenko, Vadim, Boudko, Sergei P., Barber, Mary, Mikhailova, Tatiana, Saus, Juan, Harmange, Jean-Christophe, Hudson, Billy G.
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Society for Biochemistry and Molecular Biology 2021
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Research Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC8099640/
https://ncbi.nlm.nih.gov/pubmed/33775696
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jbc.2021.100592
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