Cox8A Gene is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
{{Infobox gene
| symbol = COX8A
| name = Cytochrome C Oxidase Subunit 8A
| chromosome = 11
| locus = 11q12.2
| geneID = 9419
| omim = 601153
| ensembl = ENSG00000146205
| uniprot = P10176
| uniprot_name = COX8A
| diseases = Mitochondrial Disorders, Cardiomyopathy
| diseases_ref = DiMauro et al., 2013, Ann Neurol
}}
Cytochrome c oxidase subunit 8A (COX8A) is a nuclear-encoded mitochondrial protein that is the smallest subunit of cytochrome c oxidase (complex IV). The COX8A gene is located on chromosome 11q13.1 and encodes a protein of 69 amino acids. COX8A is expressed in tissues with high oxidative metabolism including heart, skeletal muscle, and brain. As a core component of complex IV, COX8A contributes to electron transfer from cytochrome c to molecular oxygen. In neurons, COX8A supports ATP production through oxidative phosphorylation. Mutations in COX8A cause mitochondrial complex IV deficiency and are associated with cardiomyopathy and mitochondrial encephalomyopathy.
Cytochrome c oxidase subunit 8A (COX8A) is a nuclear-encoded subunit of mitochondrial complex IV (cytochrome c oxidase). COX8A is one of the smaller subunits and is involved in the assembly and stabilization of the complex.
COX8A contributes to:
COX8A supports Complex IV function:
COX8A mutations cause mitochondrial encephalomyopathy:
COX8A deficiency leads to:
COX8A is expressed in:
The study of Cox8A Gene 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.
Malla B et al.. "Imaging and analysis of neuronal mitochondria in murine acute brain slices." Journal of neuroscience methods (2022) DOI:10.1016/j.jneumeth.2022.109558
Malla B et al.. "Teriflunomide Preserves Neuronal Activity and Protects Mitochondria in Brain Slices Exposed to Oxidative Stress." International journal of molecular sciences (2022) DOI:10.3390/ijms23031538
Rotko D et al.. "Molecular and Functional Effects of Loss of Cytochrome c Oxidase Subunit 8A." Biochemistry. Biokhimiia (2021) DOI:10.1134/S0006297921010041
Ludwig R et al.. "Investigating the Mitoprotective Effects of S1P Receptor Modulators Ex Vivo Using a Novel Semi-Automated Live Imaging Set-Up." International journal of molecular sciences (2023) DOI:10.3390/ijms25010261
Wang T et al.. "Association of cytochrome c oxidase dysfunction with amyloidosis in Alzheimer's disease and patient-derived cerebral organoids." bioRxiv : the preprint server for biology (2025) DOI:10.1101/2025.10.01.679889
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