With an alternative approach, Ruiz et al. [35] used comprehensive untargeted and targeted lipidomics in a case–control cohort of patients with Leigh syndrome French-Canadian variant (LSFC), a mitochondrial disease caused by variants in LRPPRC gene, and in mice with liver-specific inactivation of Lrpprc (H-Lrpprc−/−) unveiling a major lipid dyshomeostasis. The gene discussed is LRPPRC; the disease is Leigh syndrome.