In OMIM, four types of PCLD are included: PCLD1 (OMIM #174050) caused by heterozygous mutation in PRKCSH, PCLD2 (OMIM #617004) caused by heterozygous variant in SEC63, PCLD3 (OMIM #617874) caused by heterozygous mutant ALG8, and PCLD4 (OMIM#617875) caused by heterozygous mutation in LRP5. Among these four types, PCLD1 and PCLD2 are the most common types of PCLD. Here, PRKCSH is linked to Isolated polycystic liver disease.