SACS and channelopathy: Although multiple studies have been carried out on PKD, knowledge about the pathogenic mechanisms is limited.[24] Although the channelopathy hypothesis is prevailing in PKD, it is insufficient to explain the pathophysiology fully.[25] A recent study reported a complicated case of PKD with SACS mutation.[26] The SACS gene encodes mitochondrial protein sacsin, and variants of SACS result in defects in mitochondrial dynamics.