NEDD4-family E3 ligase dysfunction due to PKHD1/Pkhd1 defects suggests a mechanistic model for ARPKD pathobiology

Autosomal recessive polycystic kidney disease (ARPKD) is an important childhood nephropathy, occurring 1 in 20,000 live births. The major clinical phenotypes are expressed in the kidney with dilatation of the collecting ducts, systemic hypertension, and progressive renal insufficiency, and in the li...

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Publicat a:Sci Rep
Autors principals: Kaimori, Jun-ya, Lin, Cheng-Chao, Outeda, Patricia, Garcia-Gonzalez, Miguel A., Menezes, Luis F., Hartung, Erum A., Li, Ao, Wu, Guanqing, Fujita, Hideaki, Sato, Yasunori, Nakanuma, Yasuni, Yamamoto, Satoko, Ichimaru, Naotsugu, Takahara, Shiro, Isaka, Yoshitaka, Watnick, Terry, Onuchic, Luiz F., Guay-Woodford, Lisa M., Germino, Gregory G.
Format: Artigo
Idioma:Inglês
Publicat: Nature Publishing Group UK 2017
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC5552802/
https://ncbi.nlm.nih.gov/pubmed/28798345
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-017-08284-4
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