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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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Detalles Bibliográficos
Autores principales:	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.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2017
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5552802/ https://www.ncbi.nlm.nih.gov/pubmed/28798345 http://dx.doi.org/10.1038/s41598-017-08284-4

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