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 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.
仕様
Synonyms:2410004F01Rik,AU042636,COX10,COX10,Cytochrome c oxidase assembly protein,Cytochrome c oxidase subunit X,Heme A farnesyltransferase,Heme O synthase,OTTMUSP00000006085,Protoheme IX farnesyltransferase,mitochondrial,Protoheme IX farnesyltransferase,mitochondrial precursor,RP23-78H18.1
Host:Rabbit
Reactivity:Human
Applications:IHC,ELISA
Concentration:0.4mg/mL
Immunogen:Recombinant protein of human COX10
Purification Method:Affinity purification
Clonality:Polyclonal
Conjugation:Unconjugated
Buffer:PBS with 0.05% sodium azide, 50% glycerol, PH7.3
Dilution:IHC 1:50-1:200
Swissprot:Q12887
Isotype:IgG
Research Areas:Cancer, Metabolism, Signal transduction