Furthermore, in Pkd1 knockout cell lines, we show evidence of altered mitochondrial membrane potential and abnormal mitochondrial networks, and in cell lines and kidneys of Pkd1 knockout mice and in ADPKD patient samples, we show ultrastructurally abnormal mitochondria, characterized by smaller, swollen mitochondria, with less evident cristae. This evidence concerns the gene PKD1 and autosomal dominant polycystic kidney disease.