A significant increase of extracellular levels of ATP has been detected in primary cultured renal epithelial cells from both ARPKD and ADPKD patients, cyst fluid of ADPKD patients, cpk/cpk mice (a murine ARPKD model with a mutation in cystin 1 gene), as well as PCK rats (an established model for ARPKD) (Hillman et al., 2004; Palygin et al., 2018; Rangan, 2013; Schwiebert et al., 2000; Wilson et al., 1999). Here, PIK3C2A is linked to autosomal recessive polycystic kidney disease.