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). Here, DGUOK is linked to liver failure.