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 lysine for an asparagine (N204K), is identified to be responsible for cytochrome c oxidase deficiency. In addition, this gene is disrupted in patients with CMT1A (Charcot-Marie-Tooth type 1A) duplication and with HNPP (hereditary neuropathy with liability to pressure palsies) deletion.
Immunogen Information
Immunogen
Recombinant protein of human COX10
Swissprot
Q12887
Synonyms
2410004F01RikAU042636COX10COX10Cytochrome c oxidase assembly proteinCytochrome c oxidase subunit XHeme A farnesyltransferaseHeme O synthaseOTTMUSP00000006085Protoheme IX farnesyltransferasemitochondrialProtoheme IX farnesyltransferasemitochond
Gene Accession
BC000060
Applications
Reactivity
Human
Tested Applications
IHC,ELISA
Conjugation
Unconjugated
Dilution
IHC 1:50-1:200
Concentration
0.4 mg/mL
Storage Buffer
PBS with 0.05% sodium azide and 50% glycerol, PH7.4