Molecular weight (DA)
49kD
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.
Dilution rate
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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.,
Protein name
Protoheme IX farnesyltransferase mitochondrial
Other name
COX10; Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase
Fields
>>Oxidative phosphorylation;>>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors;>>Thermogenesis
Human protein sequence Database
Q12887
Mouse protein sequence database
Q8CFY5
Rat protein sequence database
Cellular localization
Mitochondrion membrane; Multi-pass membrane protein.
Contract Us
Follow Us
Location： 
Specification
成功添加到购物车