| COX4I1 Protein — Cytochrome C Oxidase Subunit 4I1 | |
|---|---|
| Gene | COX4I1 |
| UniProt | P12074 |
| PDB | 5ZVA |
| Mol. Weight | 17 kDa |
| Localization | Mitochondrial inner membrane (Complex IV) |
| Family | Cytochrome c oxidase subunit IV family |
| Diseases | Alzheimer's Disease, Parkinson's Disease, COX Deficiency |
COX4I1 encodes cytochrome c oxidase subunit IV isoform 1, a key regulatory subunit of Complex IV in the mitochondrial electron transport chain[1]. This protein plays a crucial role in oxidative phosphorylation and cellular energy production, with well-documented dysfunction in neurodegenerative diseases including Alzheimer's Disease and Parkinson's Disease[2].
COX4I1 is one of two isoforms of cytochrome c oxidase subunit IV (COX4I1 and COX4I2). COX4I1 is the dominant isoform in most tissues, including the brain, and is regulated by oxygen levels and metabolic demand[3].
Cytochrome c oxidase (Complex IV) is the terminal enzyme of the electron transport chain:
COX4I1 features:
In neurons:
Nature reviews. Mitochondrial DNA repair and neurodegeneration. Nature Reviews Neuroscience. 2010;11(5):301-315. ↩︎
DiMauro S, Schon EA. Mitochondrial respiratory-chain diseases. New England Journal of Medicine. 2003;348(26):2656-2668. ↩︎
Lin MT, Beal MF. Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature. 2006;443(7113):787-795. ↩︎