ITT1068
ITT1068
- Catalog: ITT1068
- Gene/Protein: COX10
- Product Description: Immunotag™ COX10 Polyclonal Antibody
385.0000
Price in reward points: 385
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Antibody Specification | |
Datasheet | |
Target Protein | COX10 |
Clonality | Polyclonal |
Storage/Stability | -20°C/1 year |
Application | WB,ELISA |
Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human |
Host Species | Rabbit |
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. |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Gene Name | COX10 |
Accession No. | Q12887 Q8CFY5 |
Alternate Names | COX10; Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase |
Description | COX10, heme A:farnesyltransferase cytochrome c oxidase assembly factor(COX10) Homo sapiens 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 |
Cell Pathway/ Category | Oxidative phosphorylation,Porphyrin and chlorophyll metabolism, |
Protein Expression | Brain, |
Subcellular Localization | mitochondrion,mitochondrial inner membrane,integral component of membrane,cytochrome complex, |
Protein 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., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |