製品の概要

  • 製品名Anti-DNA Polymerase gamma antibody
    DNA Polymerase gamma 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to DNA Polymerase gamma
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Rat, Human
    交差が予測される動物種: Mouse, Horse, Guinea pig, Cow, Cat, Dog, Zebrafish
  • 免疫原

    Synthetic peptide corresponding to a region within N terminal amino acids 242-291 (

    QDWQEQLVVGHNVSFDRAHIREQYLIQGSRMHFLDTMSMHMAISGLSSFQ

    ) of Rat DNA Polymerase gamma (NP_445980).

  • ポジティブ・コントロール
    • Rat lung lysate.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab116049 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 140 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

ターゲット情報

  • 機能Involved in the replication of mitochondrial DNA.
  • 関連疾患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.
  • 配列類似性Belongs to the DNA polymerase type-A family.
  • 細胞内局在Mitochondrion.
  • Information by UniProt
  • 参照データベース
  • 別名
    • DNA directed DNA polymerase gamma antibody
    • DNA polymerase subunit gamma 1 antibody
    • DNA polymerase subunit gamma-1 antibody
    • DPOG1_HUMAN antibody
    • MDP 1 antibody
    • MDP1 antibody
    • Mitochondrial DNA polymerase catalytic subunit antibody
    • Mitochondrial DNA polymerase gamma antibody
    • PEO antibody
    • POLG 1 antibody
    • POLG A antibody
    • PolG alpha antibody
    • POLG antibody
    • PolG-alpha antibody
    • POLG1 antibody
    • POLGA antibody
    • Polymerase (DNA directed) gamma antibody
    • SANDO antibody
    • SCAE antibody
    see all

Anti-DNA Polymerase gamma antibody 画像

  • Anti-DNA Polymerase gamma antibody (ab116049) at 1 µg/ml + Rat lung lysate at 10 µg

    Predicted band size : 140 kDa

Anti-DNA Polymerase gamma antibody (ab116049) 使用論文

This product has been referenced in:
  • Mancini C  et al. Genome-wide expression profiling and functional characterization of SCA28 lymphoblastoid cell lines reveal impairment in cell growth and activation of apoptotic pathways. BMC Med Genomics 6:22 (2013). WB ; Human . Read more (PubMed: 23777634) »

See 1 Publication for this product

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