Rabbit Anti-MDP1/DNA Polymerase gamma/FITC Conjugated antibody
FITC标记的DNA聚合酶γ/DNA pol γ抗体
|英文名称||Anti-MDP1/DNA Polymerase gamma/FITC|
|中文名称||FITC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|
|交叉反应||Human, Mouse, Rat, Pig,|
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|
|免 疫 原||KLH conjugated synthetic peptide derived from human DNA Polymerase gamma|
|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.|
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.
Involved in the replication of mitochondrial DNA.
Heterotrimer composed of a catalytic subunit and a homodimer of accessory subunits.
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.
Belongs to the DNA polymerase type-A family.
Entrez Gene: 613626 Cow
Entrez Gene: 145553 Human
Entrez Gene: 290230 Rat
SwissProt: Q86V88 Human
SwissProt: Q9D967 Mouse
Unigene: 19870 Rat
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
DNA的复制、重组和修复，所有这些都是基因组稳定性所必需的，需要外切核酸酶的存在。在DNA复制中，这些酶参与冈崎片段的加工，而在DNA修复中，它们起切除受损的DN**段和纠正重组失配的作用。这些核酸外切酶包括DNA聚合酶家族（DNA聚合酶）。DNA POLα、β、γ和ε参与DNA复制和修复。DNA polγ和DNA pol e是多亚基酶，DNA polγ由两个亚基组成：p125，它与滑动DNA钳位蛋白、PCNA和p50相互作用。核编码的DNA聚合酶δ是线粒体DNA复制所需的唯一DNA聚合酶。DNA POLζ在多种组织中广泛表达，介导损伤诱导的细胞机制。DNA POLθ是结合ATP的DNA聚合酶解旋酶，参与链间交联的修复。
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