Synergistic Genetic Interactions between Pkhd1 and Pkd1 Result in an ARPKD-Like Phenotype in Murine Models

Abstract excerpt

Significance Statement The lack of rapidly progressive murine models reflecting the more severe end of the spectrum of autosomal recessive polycystic kidney disease (ARPKD) inhibits progress to understanding ARPKD pathogenesis. Defects in primary cilia have been implicated in polycystic kidney disease, but their potential role is poorly understood. The authors generated and characterized new mouse and rat models...

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