| ELK.No | ES4780 |
| Product name | COX10 rabbit pAb |
| Reactivity | Human;Rat;Mouse; |
| Applications | WB;ELISA |
| Other name | COX10; Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 1352 |
| Human Swiss-Prot | Q12887 |
| Source | Rabbit |
| Isotype | IgG |
| Target | COX10 |
| Fields | >>Oxidative phosphorylation;>>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors;>>Thermogenesis |
| Gene name | COX10 |
| Protein name | Protoheme IX farnesyltransferase mitochondrial |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | |
| Mouse gene link | |
| Mouse Swiss-Prot | Q8CFY5 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| Immunogen | The antiserum was produced against synthesized peptide derived from human COX10. AA range:98-147 |
| Specificity | COX10 Polyclonal Antibody detects endogenous levels of COX10 protein. |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 49kD |
| Background | Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lys |
| Function | disease:Defects in COX10 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110]. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe multisystem disease, with onset from infancy to adulthood.,disease:Defects in COX10 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,function:Converts protoheme IX and farnesyl diphosphate to heme O.,similarity:Belongs to the ubiA prenyltransferase family., |
| Subcellular location | Mitochondrion membrane; Multi-pass membrane protein. |
| Expression | Brain, |



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