Hypertension is a well-recognized complication of autosomal recessive polycystic
kidney disease (ARPKD). The renin-angiotensin system (RAS) is a key regulator of blood
pressure; however, data on the RAS in ARPKD are limited and conflicting, showing both up-
and down-regulation. In the current study, we characterized intrarenal and systemic RAS
activation in relationship to hypertension and progressive cystic kidney disease in the
ARPKD orthologous polycystic kidney (PCK) rat. Clinical and histological measures