Murine congenital polycystic kidney disease: a model for studying development of cystic disease

GM Preminger, WE Koch, FA Fried, E McFarland… - The Journal of …, 1982 - Elsevier
The genetic, clinical and pathologic characteristics of murine congenital polycystic kidney
disease (CPK) have been 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 …