Renal injury is a third hit promoting rapid development of adult polycystic kidney disease

摘要: The 'two-hit' model is a widely accepted genetic mechanism for progressive cyst formation in autosomal dominant polycystic kidney disease. We have previously shown that adult inactivation of Pkd1 using the Mx1Cre(+) allele causes late onset focal cystic An explanation delayed appearance cysts requirement an additional independent factor, or 'third hit'. Here we show renal injury leads to massive disease same mouse line. Cysts are labeled with collecting duct/tubule marker, Lectin Dolichos biflorus Agglutinin, which correlates site Cre-mediated recombination system. 5-Bromo-2'-deoxyuridine labeling reveals cyst-lining epithelial cells comprised regenerated response injury. These data demonstrate, first time, role polycystin-1 and repair indicate constitutes hit' resulting rapid adulthood.