PKD1 and autosomal dominant polycystic kidney disease: An important question for the ADPKD field is the time point at which CFTR is activated during cystogenesis and whether this is different when the disease-causing mutation affects pkd1 or pkd2. Indeed, pkd1 mutations are associated with significantly more severe disease, triggering cystogenesis earlier in patients' lives (Cornec-Le Gall et al., 2013).