AGC2 or citrin deficiency is the most prevalent autosomal recessive inherited metabolic disease in East and East-south Asia and it is now known as a pan-ethnic disease [1,2,3,4,5,6,7,8,9], and is one of the many diseases caused by mutations in the genes encoding members of the SLC25 protein family [10,11]. Here, SLC25A13 is linked to citrin deficiency.