概述

  • 产品名称Anti-Senataxin抗体
    参阅全部 Senataxin 一抗
  • 描述
    小鼠单克隆抗体to Senataxin
  • 经测试应用适用于: ICC/IF, WB, Flow Cytmore details
  • 种属反应性
    与反应: Mouse, Human
  • 免疫原

    Recombinant fragment: VVHQDLSHIQ QPAAVVAALS SHKPPVRGEP PAASPEASTC QSKCDDPEEE LCHRREARAF SEGEQEKCGS ETHHTRRNSR WDKRTLEQED SSSKKRKL, corresponding to amino acids 2579-2677 of Human Senataxin

性能

应用

Our Abpromise guarantee covers the use of ab56984 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
ICC/IF Use a concentration of 10 µg/ml.
WB Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 303 kDa.
Flow Cyt Use 1µg for 106 cells. ab170190-Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.

靶标

  • 功能Probable helicase, which may be involved in RNA maturation (By similarity). Involved in DNA double-strand breaks damage response generated by oxidative stress.
  • 组织特异性Highly expressed in skeletal muscle. Expressed in heart, fibroblast, placenta and liver. Weakly expressed in brain and lung. Expressed in the cortex of the kidney (highly expressed in tubular epithelial cells but low expression in the glomerulus).
  • 疾病相关Defects in SETX are the cause of spinocerebellar ataxia autosomal recessive type 1 (SCAR1) [MIM:606002]; also known as ataxia-ocular apraxia 2. Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCAR1 is an autosomal recessive form associated with peripheral neuropathy and elevated serum alpha-fetoprotein, immunoglobulins and, less commonly, creatine kinase levels. Some SCAR1 patients manifest oculomotor apraxia.
    Defects in SETX are a cause of amyotrophic lateral sclerosis type 4 (ALS4) [MIM:602433]. ALS4 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of cases leading to familial forms. ALS4 is a childhood- or adolescent-onset form characterized by slow disease progression and the sparing of bulbar and respiratory muscles.
  • 序列相似性Belongs to the DNA2/NAM7 helicase family.
  • 细胞定位Nucleus > nucleoplasm. Nucleus > nucleolus. Cytoplasm. May be detected in the nucleolus only in cycling cells (By similarity). Most abundant in the nucleus. Detected in granules. Colocalized in cycling cells with FBL in the nucleolus.
  • Information by UniProt
  • 数据库链接
  • 别名
    • ALS4 antibody
    • Amyotrophic lateral sclerosis 4 protein antibody
    • AOA2 antibody
    • bA479K20.2 antibody
    • DKFZp781B151 antibody
    • FLJ12840 antibody
    • FLJ43459 antibody
    • KIAA0625 antibody
    • Probable helicase senataxin antibody
    • SCAR1 antibody
    • SEN1 homolog antibody
    • Setx antibody
    • SETX_HUMAN antibody
    see all

Anti-Senataxin antibody 图像

  • Anti-Senataxin antibody (ab56984) at 1 µg/ml + Tissue lysate prepared from human kidney at 50 µg

    Secondary
    Goat polyclonal to mouse IgG1 at 1/5000 dilution

    Predicted band size : 303 kDa
  • ab56984 at 10µg/ml staining Senataxin in Hela cells by Immunocytochemistry/ Immunofluorescence. 

  • Overlay histogram showing HEK293 cells stained with ab56984 (red line). The cells were fixed with 4% paraformaldehyde (10 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab56984, 1µg/1x106 cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG1 [ICIGG1] (ab91353, 2µg/1x106 cells) used under the same conditions. Acquisition of >5,000 events was performed. This antibody gave a positive signal in HEK293 cells fixed with 80% methanol (5 min)/permeabilized with 0.1% PBS-Tween for 20 min used under the same conditions.

Anti-Senataxin antibody (ab56984)参考文献

This product has been referenced in:
  • Nanetti L  et al. SETX mutations are a frequent genetic cause of juvenile and adult onset cerebellar ataxia with neuropathy and elevated serum alpha-fetoprotein. Orphanet J Rare Dis 8:123 (2013). WB ; Human . Read more (PubMed: 23941260) »

See 1 Publication for this product

Product Wall

Application ChIP
Sample Mouse Cell lysate - whole cell (G1E-JC4 cells)
Negative control THP promoter.
Specification G1E-JC4 cells
Detection step Real-time PCR
Type Cross-linking (X-ChIP)
Duration of cross-linking step: 40 minute(s) and 0 second(s)
Specification of the cross-linking agent: 30min 1.5mM EGS 10 min 1% HCHO
Positive control GPA promoter, Ezh2 promoter.
Username

Stefania Bottardi

Verified customer

提交于 Sep 21 2015

Thank you for the inquiry and your interest in our products. This senataxin antibody has been tested on WB against the recombinant protein and endogenous human/mouse senataxin protein. The in-house lab data indicate that ab56984 recognizes a specifi...

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"