The autosomal dominant polycystic kidney disease mutation database (PKDB) established by the Mayo Clinic lists 1243 definite pathogenic PKD1 variants out of the 2055 listed variants and 374 definite pathogenic PKD2 variants out of the 463 listed variants (http://pkdb.mayo.edu). This evidence concerns the gene PKD1 and autosomal dominant polycystic kidney disease.