• Target：
• COX10

• Fields：
• >>Oxidative phosphorylation;>>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors;>>Thermogenesis

• Gene Name：
• COX10

• Protein Name：
• Protoheme IX farnesyltransferase mitochondrial

• Human Gene Id：
• 1352

• Human Swiss Prot No：
• Q12887

• Mouse Swiss Prot No：
• Q8CFY5

• 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.

• Source：
• Polyclonal, Rabbit,IgG

• Dilution：
• WB 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：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• Other Name：
• COX10;Protoheme IX farnesyltransferase; mitochondrial;Heme O synthase

• 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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