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). This evidence concerns the gene PDHA1 and inborn mitochondrial metabolism disorder.