| Gene Symbol | MT |
| Full Name | CO1 Gene - Mitochondrially Encoded Cytochrome C Oxidase I |
| Aliases | MTCO1 |
| Function | Cytochrome b is a key component of Complex III (ubiquinol-cytochrome c reductase), which catalyzes the transfer of electrons from ubiquinol to cytochrome c. |
| GeneCards | MT |
| Human Protein Atlas | MT |
| Catalytic center | Contains heme a and heme a3, where O2 binding and reduction occur |
| Electron transfer | Receives electrons from cytochrome c and transfers to O2 |
| Proton pumping | Contributes to proton pumping across the inner membrane |
| Evolution | Encoded by mtDNA in all animals (co-evolution with nuclear-encoded subunits) |
| Hemeness | Heme a and heme a3 are unique prosthetic groups |
| Pediatric disorders | MT-CO1 mutations cause severe infantile mitochondrial disorders |
| Encephalomyopathy | Global developmental delay, lactic acidosis, cardiomyopathy |
| Heteroplasmy | Disease manifestation depends on mutant mtDNA load |
| m.7445A>G | Can cause combined LHON and sideroblastic anemia |
| Databases | GeneCardsUniProtNCBI GeneHPASTRING |
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