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Table 1 Nuclear genes involved in OXPHOS causing Leigh and Leigh-like syndrome

From: Molecular basis of Leigh syndrome: a current look

Biochemical defectAssociated nuclear genes
Complex I DeficiencyNDUFA1, NDUFA2, NDUFA9, NDUFA10, NDUFA12, NDUFS1, NDUFS2, NDUFS3, NDUFB8, NDUFS4, NDUFS7, NDUFS8, NDUFV1, NDUFV2
Complex II DeficiencySDHA
Complex III DeficiencyUQCRQ
Complex IV DeficiencyNDUFA4, COX8A
Assembly factors deficiency
 Complex INDUFAF2, NDUFAF4, NDUFAF5, NDUFAF6, C17ORF89, FOXRED1, NUBPL
 Complex IISDHAF1
 Complex IIIBCS1L, TTC19
 Complex IVSURF1, COX10, COX15, SCO2, PET100
Cofactors and biosynthesis
 Pyruvate Dehydrogenase ComplexPDHA1, PDHX, PDHB, DLAT, DLD
 Biotinidase DeficiencyBTD
 Thiamine DeficiencyTPK1, SLC19A3
 Lipoic acidLIPT1, LIAS, BOLA3
 Amino acidHIBCH, ECHS1
 Coenzyme Q10 DeficiencyPDSS2, COQ9