To investigate the pathogenic effect of mitochondrial abnormalities on cyst epithelial cells in human ADPKD, we performed in vitro studies using immortalized cyst-derived cells established from a single cyst obtained from distal cortical tubules collected from a patient with ADPKD harboring a homozygous PKD1 mutation (WT 9-12) (26) and a normal human renal cortical tubular epithelial cell (RCTEC) line derived from normal distal tubule cells (RCTEC-Dolichos biflorus agglutin [RCTEC-DBA]). Here, PKD1 is linked to autosomal dominant polycystic kidney disease.