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RabbitAnti-POLG Rabbit pAb  antibody (bs-13017R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-13017R
英文名称 POLG Rabbit pAb
中文名称 DNA聚合酶γ/DNA pol γ抗体
别    名 DNA directed DNA polymerase gamma; DNA polymerase subunit gamma 1; DNA polymerase subunit gamma-1; DPOG1_HUMAN; Mitochondrial DNA polymerase catalytic subunit; Mitochondrial DNA polymerase gamma; POLG 1; POLG A; PolG alpha; PolG-alpha; Polymerase(DNA dire  
Specific References  (1)     |     bs-13017R has been referenced in 1 publications.
111 [IF=6.24] Chen, Xubo, et al. "The role of sodium hydrosulfide in attenuating the aging process via PI3K/AKT and CaMKKβ/AMPK pathways." Redox Biology (2017).  WB ;  Rat.  222
研究领域 细胞生物  神经生物学  新陈代谢  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, Pig, )
产品应用 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 140 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human POLG: 1101-1239/1239 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 DNA replication, recombination and repair, all of which are necessary for genomic stability, require the presence of exonucleases. In DNA replication, these enzymes are involved in the processing of Okazaki fragments, whereas in DNA repair, they function to excise damaged DNA fragments and correct recombinational mismatches. These exonucleases include the family of DNA polymerases (DNA pol). DNA pol Alpha, Beta, Gamma, and epsilon are involved in DNA replication and repair. DNA pol gamma and DNA pol e are multi-subunit enzymes, with DNA pol gamma consisting of two subunits: p125, which interacts with the sliding DNA clamp protein, PCNA, and p50. The nuclear-encoded DNA pol Delta is the only DNA polymerase required for the replication of the mitochondrial DNA. DNA pol zeta is ubiquitously expressed in various tissues and mediates the cellular mechanism of damage-induced mutagenesis. DNA pol theta is a DNA polymerase-helicase that binds ATP and is involved in the repair of interstrand crosslinks.

Function:
Involved in the replication of mitochondrial DNA.

Subunit:
Heterotrimer composed of a catalytic subunit and a homodimer of accessory subunits.

Subcellular Location:
Mitochondrion.

DISEASE:
Defects in POLG are the cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 1 (PEOA1) [MIM:157640]. 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, hypogonadism, and parkinsonism.
Defects in POLG are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal recessive (PEOB) [MIM:258450]. PEOB is a severe form of progressive external ophthalmoplegia. It is clinically more heterogeneous than the autosomal dominant forms. Can be more severe.
Defects in POLG are a cause of sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO) [MIM:607459]. SANDO is a clinically heterogeneous systemic disorder with variable features resulting from mitochondrial dysfunction. It shares phenotypic characteristics with autosomal recessive progressive external ophthalmoplegia and mitochondrial neurogastrointestinal encephalopathy syndrome. The clinical triad of symptoms consists of sensory ataxic, neuropathy, dysarthria, and ophthalmoparesis.
Defects in POLG are a cause of Alpers-Huttenlocher syndrome (AHS) [MIM:203700]; also called Alpers diffuse degeneration of cerebral gray matter with hepatic cirrhosis. AHS is an autosomal recessive hepatocerebral syndrome. The typical course of AHS includes severe developmental delay, intractable seizures, liver failure, and death in childhood. Refractory seizures, cortical blindness, progressive liver dysfunction, and acute liver failure after exposure to valproic acid are considered diagnostic features. The neuropathological hallmarks of AHS are neuronal loss, spongiform degeneration, and astrocytosis of the visual cortex. Liver biopsy results show steatosis, often progressing to cirrhosis.
Defects in POLG are a cause of mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE) [MIM:603041]; also known as myoneurogastrointestinal encephalomyopathy. MNGIE is an autosomal recessive disease associated with multiple deletions of skeletal muscle mitochondrial DNA (MtDNA). It is clinically characterized by onset between the second and fifth decades of life, ptosis, progressive external ophthalmoplegia, gastrointestinal dysmotility (often pseudoobstruction), diffuse leukoencephalopathy, thin body habitus, peripheral neuropathy, and myopathy.
Defects in POLG are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Similarity:
Belongs to the DNA polymerase type-A family.

SWISS:
P54098

Gene ID:
5428

Database links:

Entrez Gene: 5428 Human

Entrez Gene: 18975 Mouse

Entrez Gene: 85472 Rat

SwissProt: P54098 Human

SwissProt: P54099 Mouse

SwissProt: Q9QYV8 Rat





产品图片
Tissue/cell: human colon cancer; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-MDP1 / DNA Polymerase gamma Polyclonal Antibody, Unconjugated(bs-13017R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: human laryngeal tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-MDP1 / DNA Polymerase gamma Polyclonal Antibody, Unconjugated(bs-13017R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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