PKD1 and autosomal dominant polycystic kidney disease: In ADPKD, the absence of PC1 led to structural and functional abnormalities in mitochondria (Ishimoto et al, 2017), disruptions in the tricarboxylic acid (TCA) cycle (Rowe et al, 2013) and a decrease in mitochondrial oxidative phosphorylation (OXPHOS) activity (Ishimoto et al, 2017).