In another female PKD1/2-negative patient (ID97.1) with ESRD at age 68 and liver transplantation for severe PCLD, we identified two rare missense variants in ALG6 (c.1465T>G, p.Phe489Val and c.1442A>G, p.Asn481Ser) encoding the ER enzyme ɑ-1,3-glucosyltransferase, an interactor of ALG8, which was previously found to be associated with a mild and atypical form of PKD and PCLD21 (Table S4, Fig. S2). The gene discussed is ALG6; the disease is Isolated polycystic liver disease.