DGUOK and Mitochondrial DNA depletion syndrome, hepatocerebral form due to DGUOK deficiency: The DGUOK genetic alteration that we chose to introduce results in a loss of function mutation, DGUOK Q170X, which is observed in patients with Mitochondrial DNA Depletion Syndrome 3 (Hepatocerebral type) (MTDPS3); the patients with this syndrome commonly die from liver failure and suffer from progressive dysfunction of neural and muscular tissues (Mandel et al., 2001).