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DNA pol γ2 Polyclonal Antibody
DNA pol γ2 Polyclonal Antibody
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DNA pol γ2 Polyclonal Antibody
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经销商客户: ¥214.5
实验室客户: ¥292.5
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商品属性

Target:DNA pol γ2

Gene Name:POLG2

Protein Name:DNA polymerase subunit gamma-2 mitochondrial

Human Gene Id:11232

Human Swiss Prot No:Q9UHN1

Mouse Swiss Prot No:Q9QZM2

Immunogen:The antiserum was produced against synthesized peptide derived from human POLG2. AA range:291-340

Specificity:DNA pol γ2 Polyclonal Antibody detects endogenous levels of DNA pol γ2 protein.

Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source:Polyclonal, Rabbit,IgG

Dilution:IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200

Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration:1 mg/ml

Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name:POLG2;MTPOLB;DNA polymerase subunit gamma-2; mitochondrial;DNA polymerase gamma accessory 55 kDa subunit;p55;Mitochondrial DNA polymerase accessory subunit;MtPolB;PolG-beta

Molecular Weight(Da):55kD

Background: This gene encodes the processivity subunit of the mitochondrial DNA polymerase gamma. The encoded protein forms a heterotrimer containing one catalytic subunit and two processivity subunits. This protein enhances DNA binding and promotes processive DNA synthesis. Mutations in this gene result in autosomal dominant progressive external ophthalmoplegia with mitochondrial DNA deletions.[provided by RefSeq, Sep 2009],

Function:catalytic activity:Deoxynucleoside triphosphate + DNA(n) = diphosphate + DNA(n+1).,disease:Defects in POLG2 are the cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 4 (PEOA4) [MIM:610131]. Progressive external ophthalmoplegia is characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged-red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadis

Subcellular Location:Mitochondrion.

Expression: Cerebellum,Cervix,Ovary,

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