产品名称
COQ2 Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/27235
Human Swissprot No.
Q96H96
Human Swissprot Link
https://www.uniprot.org/uniprotkb/Q96H96/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/71883
Mouse Swissprot No.
Q66JT7
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q66JT7
Rat Gene Link
https://www.uniprot.org/uniprot/498332
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/Q499N4
免疫原
Synthesized peptide derived from human COQ2 AA range: 160-210
特异性
This antibody detects endogenous levels of COQ2 at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant. This enzyme, which is part of the coenzyme Q10 pathway, catalyzes the prenylation of parahydroxybenzoate with an all-trans polyprenyl group. Mutations in this gene cause coenzyme Q10 deficiency, a mitochondrial encephalomyopathy, and also COQ2 nephropathy, an inherited form of mitochondriopathy with primary renal involvement. [provided by RefSeq, Oct 2009],
组织表达
Widely expressed. Present in all of the tissues tested. Expressed at higher level in skeletal muscle, adrenal glands and the heart.
细胞定位
Mitochondrion inner membrane ; Multi-pass membrane protein ; Matrix side .
功能
disease:Defects in COQ2 are a cause of coenzyme Q10 deficiency [MIM:607426]. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy.,function:Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB.,pathway:Cofactor biosynthesis; ubiquinone biosynthesis.,similarity:Belongs to the ubiA prenyltransferase family.,tissue specificity:Widely expressed. Present in all of the tissues tested. Expressed at higher level in skeletal muscle, adrenal glands and the heart.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
