Abstract:Background and Aims
Autosomal recessive polycystic kidney disease is caused by mutations in PKHD1 encoding FPC, and is characterized by severe renal cystogenesis in neonates, yet mouse models do not fully recapitulate the human phenotype. Indeed, even the Pkhd1 null allele does not cause renal cystogenesis in the mouse. Several cleavage products of FPC are reported yet their function remains unknown. The aim of this study was to determine the function of the FPC cleavage products and their ef… Show more
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