Murine Congenital Polycystic Kidney Disease: A Model for Studying Development of Cystic Disease

Abstract

The genetic, clinical and pathologic characteristics of murine congenital polycystic kidney disease (CPK) were examined in mutant mice highly congenic with C57BL/6J inbred strains. This CPK defect appears to resemble closely human infantile polycystic kidney disease in its pattern of inheritance, clinical course and appearance of the kidney in the severely affected animal. Histologic studies of early postnatal animals suggest that cystic alterations may occur first in the proximal tubules and secondarily affect the collecting ducts. The CPK mouse mutant should prove useful in further investigations into the early morphologic alterations and progression of cystic disease.