Mutations in SUCLA2 (coding for succinate-CoA ligase, beta subunit) and SUCLG1 (coding for succinate-CoA ligase, alpha subunit) cause an encephalomyopathic form of infantile MDS, but mutations in SUCLG1 can also cause a severe disorder characterized by antenatal dysmorphisms, neonatal metabolic crisis, and early death [47]. The gene discussed is SUCLG1; the disease is myelodysplastic syndrome.