PKD1 and autosomal dominant polycystic kidney disease: Our recentin vitro study 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 calcium-sensing receptor increases cytosolic calcium, reduces intracellular cAMP and mTOR activity118, and rescues defective ATP mitochondrial production119, reversing the principal ADPKD dysregulations.