Article
ER stress and basement membrane defects combine to cause glomerular and tubular renal disease resulting from Col4a1 mutations in mice.
Disease models & mechanisms - 1 Feb 2016
Jones Frances E, Bailey Matthew A, Murray Lydia S, Lu Yinhui, McNeilly Sarah, Schlötzer-Schrehardt Ursula, Lennon Rachel, Sado Yoshikazu, Brownstein David G, Mullins John J, Kadler Karl E, Van Agtmael Tom
Abstract excerpt
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 are unknown. We show that Col4a1 mutations in mice cause hypotension and renal disease, including proteinuria and...
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