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| Cat.No | ACP05859 | Target Name | COX14 |
|---|---|---|---|
| Target Synonyms | COX14; C12orf62; Cytochrome c oxidase assembly protein COX14 | Form | Lyophilized powder |
| Expression System | Custom Production. Please inquire and provide the desire expression system. | Protein Length | Partial |
| Purity | >85% (SDS-PAGE) | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | Q96I36 |
|---|
Uniprot Id
Q96I36
Target Species
Human
Target Name
COX14
Target Full Name
Cytochrome c oxidase assembly protein COX14
Target Function
Core component of the MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase complex) complex, that regulates cytochrome c oxidase assembly. Requires for coordination of the early steps of cytochrome c oxidase assembly with the synthesis of MT-CO1.
Target Involvement
Defects in COX14 may be a cause of a mitochondrial disorder presenting with severe congenital lactic acidosis and dysmorphic features associated with a COX assembly defect. Other features include brain hypertrophy, diffuse alteration of the white-matter myelination, and numerous cavities in the parieto-occipital region, brainstem, and cerebellum, as well as hepatomegaly, hypertrophic cardiomyopathy, renal hypoplasia, and adrenal-gland hyperplasia.
Target Subcellular Location
Mitochondrion membrane; Single-pass membrane protein.
Target Synonyms
COX14; C12orf62; Cytochrome c oxidase assembly protein COX14
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