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

• Main Authors: Frances E. Jones (Author), Matthew A. Bailey (Author), Lydia S. Murray (Author), Yinhui Lu (Author), Sarah McNeilly (Author), Ursula Schlötzer-Schrehardt (Author), Rachel Lennon (Author), Yoshikazu Sado (Author), David G. Brownstein (Author), John J. Mullins (Author), Karl E. Kadler (Author), Tom Van Agtmael (Author)
• Format: Book
• Published: The Company of Biologists, 2016-02-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...