Mutations in deoxyguanosine kinase (DGUOK), mitochondrial inner membrane protein MPV17, polymerase catalytic subunit (POLG), succinate-CoA ligase GDP/ADP-forming subunit alpha (SUCLG1), twinkle MtDNA helicase (TWNK) and transcription factors A (TFAM) have been associated with hepatocerebral MDDS, with acute liver failure in infancy and hypoglycemia. The gene discussed is DGUOK; the disease is acute liver failure.