DGUOK and liver disorder: Despite the heterogeneity of clinical phenotypes, most MTDPS3 patients suffer from hypoglycemia, lactic acidosis, and progressive liver disease and commonly die from liver failure in infancy or early childhood (Mandel et al., 2001; Salviati et al., 2002; Mancuso et al., 2005; Dimmock et al., 2008b).