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Article

Collagen IVα345 dysfunction in glomerular basement membrane diseases. III. A functional framework for α345 hexamer assembly.

The Journal of biological chemistry - 1 Jan 2000

Pedchenko Vadim, Boudko Sergei P, Barber Mary, Mikhailova Tatiana, Saus Juan, Harmange Jean-Christophe, Hudson Billy G

Abstract excerpt

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 cause glomerular basement membrane disease by exploring the mechanism of the hexamer assembly. Chloride ions...

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Identifiers

PMID: 33775696
DOI: 10.1016/j.jbc.2021.100592
PMCID: PMC8099640

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Collagen IVα345 dysfunction in glomerular basement membrane diseases. III. A functional framework for α345 hexamer assembly.PMID 33775696Read ↗

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