A novel model of autosomal recessive polycystic kidney questions the role of the Fibrocystin C-terminus in disease mechanism

Autosomal recessive polycystic kidney disease (OMIM 263200) is a serious condition of the kidney and liver caused by mutations in a single gene, PKHD1. This gene encodes Fibrocystin/Polyductin (FPC, PD1), a large protein shown by in vitro studies to undergo Notch-like processing. Its cytoplasmic tai...

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Detalhes bibliográficos
Publicado no:Kidney Int
Main Authors: Outeda, Patricia, Menezes, Luis, Hartung, Erum A., Bridges, Stacey, Zhou, Fang, Zhu, Xianjun, Xu, Hangxue, Huang, Qiong, Yao, Qin, Qian, Feng, Germino, Gregory G, Watnick, Terry
Formato: Artigo
Idioma:Inglês
Publicado em: 2017
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6005173/
https://ncbi.nlm.nih.gov/pubmed/28729032
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.kint.2017.04.027
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