Finally, MEGDEL syndrome (3-methylglutaconic aciduria, deafness, encephalopathy, and Leigh-like syndrome) is caused by variants in the serine active site containing 1 gene (SERAC1) [124], the protein product of which is implicated in the remodeling of PG, an important precursor of CL (see Figure 3 in the main text). The gene discussed is SERAC1; the disease is Encephalopathy.