| 18763 | O08852 | PKD1_MOUSE | Polycystin-1 (Autosomal dominant polycystic kidney disease 1 protein homolog) | DISRUPTION PHENOTYPE | Knockin mice expressing non-cleavable PKD1 show a hypomorphic phenotype. They are viable, show rapid cystic dilation in renal collecting duct and distal convoluted tubule, but not in the proximal portion of the nephron, during the postnatal period, and die with severe uremia, mostly at 3 weeks of age. Additionally, they show dilation of the common bile duct and intrahepatic biliary ducts, but develop a normal pancreas within their life span. {ECO:0000269|PubMed:18003909}. |