AAV-mediated hepatic ETHE1 gene expression restores sulphur dioxygenase activity in a murine model of ethylmalonic encephalopathy, correcting biochemical abnormalities within liver, muscle, and brain; and increasing survival from a few weeks to more than 6 months (Di Meo et al., 2012). Here, ETHE1 is linked to ethylmalonic encephalopathy.