重组Anti-POLG抗体[EPR7296] (ab128899)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR7296] to POLG
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
Related conjugates and formulations
概述
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产品名称
Anti-POLG抗体[EPR7296]
参阅全部 POLG 一抗 -
描述
兔单克隆抗体[EPR7296] to POLG -
宿主
Rabbit -
经测试应用
适用于: WBmore details -
种属反应性
与反应: Mouse, Rat, Human -
免疫原
Synthetic peptide within Human POLG aa 1150-1200. The exact sequence is proprietary.
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阳性对照
- MCF7, HepG2, 293T, RAW 264.7 and C6 cell lysates.
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常规说明
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Avoid freeze / thaw cycle. Stable for 12 months at -20°C. -
解离常数(KD)
KD = 1.50 x 10 -11 M Learn more about KD -
存储溶液
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: PBS, 40% Glycerol, 0.05% BSA -
Concentration information loading...
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纯度
Protein A purified -
克隆
单克隆 -
克隆编号
EPR7296 -
同种型
IgG -
研究领域
相关产品
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Alternative Versions
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Compatible Secondaries
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Isotype control
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Positive Controls
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab128899于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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WB |
1/1000 - 1/10000. Detects a band of approximately 140 kDa (predicted molecular weight: 140 kDa).
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说明 |
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WB
1/1000 - 1/10000. Detects a band of approximately 140 kDa (predicted molecular weight: 140 kDa). |
靶标
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功能
Involved in the replication of mitochondrial DNA. -
疾病相关
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. -
序列相似性
Belongs to the DNA polymerase type-A family. -
细胞定位
Mitochondrion. - Information by UniProt
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数据库链接
- Entrez Gene: 5428 Human
- Entrez Gene: 18975 Mouse
- Entrez Gene: 85472 Rat
- Omim: 174763 Human
- SwissProt: P54098 Human
- SwissProt: P54099 Mouse
- SwissProt: Q9QYV8 Rat
- Unigene: 706868 Human
see all -
别名
- DNA directed DNA polymerase gamma antibody
- DNA polymerase subunit gamma 1 antibody
- DNA polymerase subunit gamma-1 antibody
see all
图片
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All lanes : Anti-POLG antibody [EPR7296] (ab128899) at 1/5000 dilution (purified)
Lane 1 : HEK-293T (human embryonic kidney) whole cell lysate
Lane 2 : HepG2 (Human liver hepatocellular carcinoma cell line) whole cell lysate
Lysates/proteins at 20 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution
Predicted band size: 140 kDa
Observed band size: 140 kDaBlocking and diluting buffer: 5% NFDM/TBST.
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All lanes : Anti-POLG antibody [EPR7296] (ab128899) at 1/1000 dilution (purified)
Lane 1 : RAW 264.7 (Mouse macrophage cell line transformed with Abelson murine leukemia virus) whole cell lysate
Lane 2 : C6 (Rat glial tumor cell line) whole cell lysate
Lysates/proteins at 20 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution
Predicted band size: 140 kDa
Observed band size: 140 kDaBlocking and diluting buffer: 5% NFDM /TBST.
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All lanes : Anti-POLG antibody [EPR7296] (ab128899) at 1/1000 dilution (unpurified)
Lane 1 : MCF7 cell lysate
Lane 2 : 293T cell lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution
Predicted band size: 140 kDa
数据表及文件
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SDS download
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Datasheet download
文献 (23)
ab128899 被引用在 23 文献中.
- Sidarala V et al. Mitofusin 1 and 2 regulation of mitochondrial DNA content is a critical determinant of glucose homeostasis. Nat Commun 13:2340 (2022). PubMed: 35487893
- Hangas A et al. Top3α is the replicative topoisomerase in mitochondrial DNA replication. Nucleic Acids Res 50:8733-8748 (2022). PubMed: 35904803
- D'Acunzo P et al. Isolation of mitochondria-derived mitovesicles and subpopulations of microvesicles and exosomes from brain tissues. Nat Protoc 17:2517-2549 (2022). PubMed: 35962195
- Xiao J et al. PGC-1? Mediated-EXOG, a Specific Repair Enzyme for Mitochondrial DNA, Plays an Essential Role in the Rotenone-Induced Neurotoxicity of PC12 Cells. J Mol Neurosci N/A:N/A (2021). PubMed: 33515431
- Lee Y et al. De Novo Development of mtDNA Deletion Due to Decreased POLG and SSBP1 Expression in Humans. Genes (Basel) 12:N/A (2021). PubMed: 33671400