Mutations in the gene encoding AGC2 are known to cause two liver diseases—neonatal intrahepatic cholestasis by citrin deficiency (NICCD, OMIM 605814) and type-II citrullinemia (CTLN2, OMIM 215700)—mainly due to lack of cytosolic NADH oxidation and impaired urea cycle functioning [36]. Here, SLC25A13 is linked to citrullinemia type II.