PKD1 and autosomal dominant polycystic kidney disease: For example, autosomal dominant polycystic kidney disease (ADPKD), one of the most common human monogenic diseases affecting ca. 1:1000 live births, can be caused by mutations in PKD1 or PKD2 encoding the cilium-localized transmembrane proteins polycystin-1 (PC1) and polycystin-2 (PC2), respectively, which form a heterodimeric calcium-permeable nonselective cation channel complex essential for tubular differentiation, polarity and diameter in the kidney (Cantero and Cantiello, 2022; Ma et al, 2017; Pazour et al, 2002; Yoder et al, 2002).