The mitochondrial disease Leigh Syndrome is caused by mutations in more than 75 nuclear- or mitochondrial-encoded genes such as several subunits of Complex I (e.g., NDUFS4), Complex IV (e.g., COX10), assembly factors (e.g., SURF1), metabolic enzymes (PDHA1), and transport proteins (SLC19A3) among others (Stenton and Prokisch, 2020). The gene discussed is PDHA1; the disease is Leigh syndrome.