Cytochrome c oxidase (COX), the terminal enzyme of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. It 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 be involved in the regulation and assembly of the complex. This nuclear gene encodes subunit VIb. Three pseudogenes COX6BP-1, COX6BP-2 and COX6BP-3 have been found on chromosomes 7, 17 and 22q13.1-13.2, respectively. (provided by RefSeq)
Immunogen
COX6B1 (AAH01015, 1 a.a. ~ 86 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Monoclonal Anti-COX6B1 antibody produced in mouse is suitable for capture ELISA, indirect ELISA and western blot applications.
Biochem/physiol Actions
COX6B1 (cytochrome c oxidase subunit VIb polypeptide 1) is involved in the regulation of mitochondrial biogenesis. Itis involved in the transport of electron from reduced cytochrome C to molecular oxygen. In muscles, COX gene consists of striated muscle-specific regulatory motifs such as E-box, CArG, and MEF2 at the proximal promoter regions. The large hydrophobic catalytic subunit acts in the electron transfer whereas the small unit is responsible for regulation and assembly of the complex. In mammals, it exists as a monomer with 13 subunits but in the active state serves as a dimer in vivo. Deficiency of COX6B1 causes mitochondrial diseases such as encephalomyopathy, hydrocephalus and hypertropic cardiomyopathy.
European journal of human genetics : EJHG, 23(2), 159-164 (2014-05-02)
Isolated cytochrome c oxidase (COX) deficiency is a prevalent cause of mitochondrial disease and is mostly caused by nuclear-encoded mutations in assembly factors while rarely by mutations in structural subunits. We hereby report a case of isolated COX deficiency manifesting
Progress in nucleic acid research and molecular biology, 61, 309-344 (1998-09-30)
Cytochrome c Oxidase (COX) is the terminal component of the bacterial as well as the mitochondrial respiratory chain complex that catalyzes the conversion of redox energy to ATP. In eukaryotes, the oligomeric enzyme is bound to mitochondrial innermembrane with subunits
American journal of human genetics, 82(6), 1281-1289 (2008-05-24)
Cytochrome c oxidase (COX) deficiency, one of the most common respiratory-chain defects in humans, has been associated with mutations in either mitochondrial DNA genes or nucleus-encoded proteins that are not part in but promote the biogenesis of COX. Mutations of
Questions
Reviews
★★★★★ No rating value
Active Filters
Our team of scientists has experience in all areas of research including Life Science, Material Science, Chemical Synthesis, Chromatography, Analytical and many others.