ER stress and basement membrane defects combine to cause glomerular and tubular renal disease resulting from Col4a1 mutations in mice

ER stress and basement membrane defects combine to cause glomerular and tubular renal disease resulting from Col4a1 mutations in mice

Collagen IV is a major component of basement membranes, and mutations in COL4A1, which encodes collagen IV alpha chain 1, cause a multisystemic disease encompassing cerebrovascular, eye and kidney defects. However, COL4A1 renal disease remains poorly characterized and its pathomolecular mechanisms a...

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Bibliographic Details
Main Authors: Frances E. Jones, Matthew A. Bailey, Lydia S. Murray, Yinhui Lu, Sarah McNeilly, Ursula Schlötzer-Schrehardt, Rachel Lennon, Yoshikazu Sado, David G. Brownstein, John J. Mullins, Karl E. Kadler, Tom Van Agtmael
Format: Article
Language:English
Published: The Company of Biologists 2016-02-01
Series:Disease Models & Mechanisms
Subjects:
Collagen IV
Extracellular matrix
Endoplasmic reticulum stress
Basement membrane
Kidney disease
Online Access:http://dmm.biologists.org/content/9/2/165

Internet

http://dmm.biologists.org/content/9/2/165

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