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货号： bs-13017R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-13017R-PE-Cy7
- 英文名称
- Anti-MDP1/DNA Polymerase gamma/PE-Cy7
- 中文名称
- PE-Cy7标记的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; MDP 1; MDP1; Mitochondrial DNA polymerase catalytic subunit; Mitochondrial DNA polymerase gamma; PEO; POLG 1; POLG A; PolG alpha; POLG; PolG-alpha; POLG1; POLGA; Polymerase (DNA directed) gamma; SANDO; SCAE.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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, Mouse, Rat, Pig,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 140kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human DNA Polymerase gamma
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
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.
Database links:Entrez Gene: 613626Cow
Entrez Gene: 145553Human
Entrez Gene: 290230Rat
SwissProt: Q86V88Human
SwissProt: Q9D967Mouse
Unigene: 19870Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.