概述

  • 产品名称Anti-Aprataxin抗体
    参阅全部 Aprataxin 一抗
  • 描述
    兔多克隆抗体to Aprataxin
  • 经测试应用适用于: ELISA, WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Horse, Chicken, Guinea pig, Cow, Cat, Dog, Pig
  • 免疫原

    Synthetic peptide: EMVQEAGRVT VRDGMPELLK LPLRCHECQQ LLPSIPQLKE HLRKHWTQ, corresponding to amino acids 295-342 of Human Aprataxin

  • 阳性对照
    • Human kidney tissue sections and NCI-H226 cell lysate

性能

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应用

Our Abpromise guarantee covers the use of ab66861 in the following tested applications.

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

应用 Ab评论 说明
ELISA Use at an assay dependent concentration.

ELISA titre using peptide based assay 1:1562500.

WB Use a concentration of 2.5 µg/ml. Detects a band of approximately 39 kDa (predicted molecular weight: 39 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
IHC-P Use a concentration of 4 - 8 µg/ml.

靶标

  • 功能DNA-binding protein involved in single-strand DNA break repair, double-strand DNA break repair and base excision repair. Resolves abortive DNA ligation intermediates formed either at base excision sites, or when DNA ligases attempt to repair non-ligatable breaks induced by reactive oxygen species. Catalyzes the release of adenylate groups covalently linked to 5'-phosphate termini, resulting in the production of 5'-phosphate termini that can be efficiently rejoined. Also able to hydrolyze adenosine 5'-monophosphoramidate (AMP-NH(2)) and diadenosine tetraphosphate (AppppA), but with lower catalytic activity.
  • 组织特异性Widely expressed. In brain, it is expressed in the posterior cortex, cerebellum, hippocampus and olfactory bulb. Isoform 1 is highly expressed in the cerebral cortex and cerebellum, compared to isoform 2.
  • 疾病相关Defects in APTX are the cause of ataxia-oculomotor apraxia syndrome (AOA) [MIM:208920]. AOA is an autosomal recessive syndrome characterized by early-onset cerebellar ataxia, oculomotor apraxia, early areflexia and late peripheral neuropathy.
    Defects in APTX are a cause of coenzyme Q10 deficiency (COQ10D) [MIM:607426]. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy.
  • 序列相似性Contains 1 C2H2-type zinc finger.
    Contains 1 FHA-like domain.
    Contains 1 HIT domain.
  • 结构域The histidine triad, also called HIT motif, forms part of the binding loop for the alpha-phosphate of purine mononucleotide.
    The FHA-like domain mediates interaction with NCL; XRCC1 and XRCC4.
    The HIT domain is required for enzymatic activity.
    The C2H2-type zinc finger mediates DNA-binding.
  • 细胞定位Nucleus > nucleoplasm. Nucleus > nucleolus. Upon genotoxic stress, colocalizes with XRCC1 at sites of DNA damage. Colocalizes with MDC1 at sites of DNA double-strand breaks. Interaction with NCL is required for nucleolar localization.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AOA 1 antibody
    • AOA antibody
    • AOA1 antibody
    • Aprataxin antibody
    • Aprataxin homolog antibody
    • Aptx antibody
    • APTX_HUMAN antibody
    • Ataxia 1 early onset with hypoalbuminemia antibody
    • Ataxia1 early onset with hypoalbuminemia antibody
    • AXA 1 antibody
    • AXA1 antibody
    • EAOH antibody
    • EOAHA antibody
    • FHA HIT antibody
    • FHA-HIT antibody
    • FLJ20157 antibody
    • Forkhead associated domain histidine triad like antibody
    • Forkhead associated domain histidine triad like protein antibody
    • Forkhead-associated domain histidine triad-like protein antibody
    • MGC1072 antibody
    see all

Anti-Aprataxin antibody 图像

  • Anti-Aprataxin antibody (ab66861) at 2.5 µg/ml + NCI-H226 cell lysate at 10 µg

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 39 kDa
    Observed band size : 39 kDa
    Gel concentration: 12%
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human kidney tissue labelling Aprataxin with ab66861 at 4-8µg/ml. Arrows indicate positively labelled epithelial cells of the renal tubules. Magnification: 400X.

Anti-Aprataxin antibody (ab66861)参考文献

ab66861 has not yet been referenced specifically in any publications.

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