Development of autosomal recessive polycystic kidney disease in BALB/c-cpk/cpk mice

JL Ricker, II VINCENT H GATTONE… - Journal of the …, 2000 - journals.lww.com
Autosomal recessive polycystic kidney disease (ARPKD) is a rare but devastating inherited
disease in humans. Various strains of mice that are homozygous for the cpk gene display
renal pathology similar to that seen in human ARPKD. The PKD progresses to renal
insufficiency, azotemia, and ultimately a uremic death by approximately 3 wk of age. This
study characterizes PKD in mice that are homozygous for the cpk gene on a BALB/c inbred
mouse background. The BALB/c-cpk/cpk murine model displays renal as well as extrarenal …