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Polycystic kidney disease: a Hippo connection

Mutations in PKD1 and PKD2 are the leading cause of autosomal dominant polycystic kidney disease (ADPKD). In this issue of Genes & Development, a report by Cai and colleagues (pp. 781–793) reveals new insight into the molecular basis by which PKD1 deficiency leads to cystic kidney pathogenesis....

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Detalles Bibliográficos
Autores principales: Ma, Shenghong, Guan, Kun-Liang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cold Spring Harbor Laboratory Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6049516/
https://www.ncbi.nlm.nih.gov/pubmed/29921661
http://dx.doi.org/10.1101/gad.316570.118
Descripción
Sumario:Mutations in PKD1 and PKD2 are the leading cause of autosomal dominant polycystic kidney disease (ADPKD). In this issue of Genes & Development, a report by Cai and colleagues (pp. 781–793) reveals new insight into the molecular basis by which PKD1 deficiency leads to cystic kidney pathogenesis. By using extensive mouse genetic analyses coupled with in vitro cystic assays, the investigators delineate a RhoA–YAP–c-Myc signaling axis as a key downstream from PKD1 deficiency in ADPKD pathogenesis. Their findings provide evidence that the Hippo pathway could be a potential target for treating ADPKD.