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COQ2 mouse mAb

BYmab-08748

  • 20UL ¥460 50UL ¥945 100UL ¥1350
  • 货期: 3个工作日

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宿主
反应性
应用
WB
分子量(DA)
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
来源
Monoclonal, Mouse,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB 1:500-2000
纯化工艺(Immunogen)
The antibody was affinity-purified from mouse serum by affinity-chromatography using specific immunogen.
浓度
1 mg/ml
背景(Background)
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],
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 glan
基因名称(Gene Name)
COQ2 CL640
蛋白名称
COQ2
简称
COQ2
其他名称
Fields
>>Ubiquinone and other terpenoid-quinone biosynthesis;>>Metabolic pathways;>>Biosynthesis of cofactors
人基因ID
人蛋白质序列数据库
小鼠基因ID
小鼠蛋白质序列数据库
大鼠基因ID
大鼠蛋白质序列数据库
细胞定位
Mitochondrion inner membrane ; Multi-pass membrane protein ; Matrix side .
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.
储存(Storage)
-20°C/1 year

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COQ2 mouse mAb

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