Mutations in LIPT1 were found to cause some inherited metabolic disorders, such as a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase and a fatal disease related to a specific lipoylation defect of the 2-ketoacid dehydrogenase complexes (Soreze et al., 2013; Tort et al., 2014). Here, LIPT1 is linked to metabolic disease.