COX10 Polyclonal Antibody

Ab type

Primary antibody

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. COX10 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. COX10, heme A: farnesyltransferase cytochrome c oxidase assembly factor 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.

Reactivity

Human

Clonality

Polyclonal

Host

Rabbit

Isotype

IgG

Immunogen

The antiserum was produced against synthesized peptide derived from human COX10. AA range:98-147

Storage Stability

-20°C for one year

Concentration

1 mg/ml

MolecularWeight_Da

48882

synonyms

COX10; Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase