| Full Name | Mitochondrially Encoded Cytochrome C Oxidase I |
|---|---|
| Chromosomal Location | mitochondrial genome (MT:5904-7445) |
| NCBI Gene ID | 4513 |
| OMIM | 516030 |
| Ensembl ID | ENSG00000198804 |
| UniProt | P00395 |
| Associated Diseases | Leber's Hereditary Optic Neuropathy, Cytochrome C Oxidase Deficiency, Sideroblastic Anemia |
MT-CO1 (Mitochondrially Encoded Cytochrome C Oxidase I) is the largest subunit of Complex IV (Cytochrome c Oxidase) and forms the catalytic core of the enzyme[1]. It is essential for the final step of the electron transport chain where electrons are transferred to oxygen[2].
The MT-CO1 gene encodes a 513-amino acid transmembrane protein:
MT-CO1 is expressed in all aerobic tissues:
The study of Mt Co1 Gene Mitochondrially Encoded Cytochrome C Oxidase I has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
Hunte C, et al. Structure of the cytochrome bc1 complex. Annual Review of Biochemistry. 2010;79:1-34. doi:10.1146/annurev.biochem.78.081307.095057. ↩︎
Zhang Z, et al. Cytochrome c oxidase: structure and mechanism. Current Opinion in Structural Biology. 2006;16(4):465-472. doi:10.1016/j.sbi.2006.06.005. ↩︎
Rich PR, Maréchal A. The mitochondrial respiratory chain. Essays in Biochemistry. 2010;47:1-23. doi:10.1042/bse0470001. ↩︎
Cooper CE, et al. Cytochrome c oxidase deficiency and neurodegeneration. Mitochondrion. 2007;7(1-2):2-13. doi:10.1016/j.mito.2006.11.016. ↩︎