The disruption of primary cilia or mutations in cilia-associated proteins results in renal epithelial proliferation and cyst growth in animal models and human genetic diseases that are classified as ciliopathies (Hildebrandt et al., 2011), for instance characteristic of the development of the autosomal recessive polycystic kidney disease (APKD) [(Singla and Reiter, 2006); (Kolb and Nauli, 2008)]. Here, CYS1 is linked to ciliopathy.