| Complex | Name | OMIM | Function | Chromosome | Inheritance | Clinical Phenotype | References |
| I | NDUFS1 | 157655 | IP fraction | 2q33-q34 | AR | Leigh Syndrome | (17) |
| I | NDUFS2 | 602985 | IP fraction | 1q23 | AR | Encephalopathy, Cardiomyopathy | (18) |
| I | NDUFS3 | 603846 | IP fraction | 11p11.11 | AR | Leigh Syndrome | (11) |
| I | NDUFS4 | 602694 | IP fraction | 5q11.1 | AR | Leigh Syndrome | (23) |
| I | NDUFS6 | 603848 | IP fraction | 5pter-p15.33 | AR | Fatal Infantile Lactic Acidosis | (5,12) |
| I | NDUFS7 | 601825 | HP fraction | 19p13.3 | AR | Leigh Syndrome | (21) |
| I | NDUFS8 | 602141 | HP fraction | 11q13 | AR | Leigh Syndrome | (13,22) |
| I | NDUFB3 | 603839 | HP fraction | 2q31.3 | AR | Fatal Infantile Lactic Acidosis | (1) |
| I | NDUFV1 | 161015 | FP fraction | 11q13 | AR | Leigh Syndrome | (21) |
| I | NDUFV2 | 600532 | FP fraction | 18p11 | AR | Cardiomyopathy, hypotonia, encephalopathy | (14) |
| I | NDUFA1 | 300078 | HP fraction | Xq24 | X | Leigh Syndrome, Progressive neurodegenerative disorder | (4, 10) |
| I | NDUFA2 | 602137 | HP fraction | 5q31.2 | AR | Leigh Syndrome | (8) |
| I | NDUFA10 | 603835 | HP fraction | 2q37.3 | AR | Leigh Syndrome | (2) |
| I | NDUFA11 | 612638 | IP fraction | 19p13.3 | AR | Fatal Infantile Lactic Acidosis Encephalocardiomyopathy | (7) |
| II | SDH-A | 600857 | FP subunit | 5p15 | AR | Leigh Syndrome | (24) |
| II | SDH-B | 185470 | IP subunit | 1p36.1-p35 | AD | Phaeochromocytoma and paraganglioma | (16) |
| II | SDH-C | 602413 | Membrane subunit | 1q21 | AD | Autosomal dominant paraganglioma type 3 | (20) |
| II | SDH-D | 602690 | Membrane subunit | 11q23 | AD | Autosomal dominant paraganglioma type 1, Pheochromocytoma | (19) |
| III | UQCRB | 191330 | electron transfer | 8q22 | AR | Hypoglycemia, Lactic acidosis | (15) |
| III | UQCRQ | 612080 | electron transfer | 5q31.1 | AR | Severe neurological phenotype | (6) |
| IV | COX6B1 | 124089 | Cytochrome oxidase activity | 19q13.1 | AR | Encephalomyopathy | (9) |
| V | ATP5E | 606153 | ATPase activity | 20q13.3 | AR | Lactic acidosis, mental retardation, peripheral neuropathy | (3) |
Abbreviations
FP: Complex I or II flavoprotein fraction; IP: Complex I or II iron-sulfur protein fraction; HP: Complex I hydrophobic protein; AR: Autosomal Recessive; AD: Autosomal Dominant; X: X-linked
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