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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...
Autores principales: | 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 |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Company of Biologists Ltd
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4770143/ https://www.ncbi.nlm.nih.gov/pubmed/26839400 http://dx.doi.org/10.1242/dmm.021741 |
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