CASR and autosomal dominant polycystic kidney disease: Specifically, we recently demonstrated that in human conditionally immortalized proximal tubular epithelial cells silenced for PKD1 (ciPTEC-PC1KD) or generated from a patient with ADPKD1 (ciPTEC-PC1Pt), selective activation of the CaSR increases cytosolic Ca2+, reduces intracellular cAMP and mTOR activity [24], and rescues defective ATP mitochondrial production [25], reversing the principal ADPKD dysregulations.