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,