Overview

  • Product name

  • Description

    Rabbit polyclonal to Senataxin
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

    Reacts with: Mouse
    Predicted to work with: Rat, Human
  • Immunogen

    Synthetic peptide within Human Senataxin aa 345-395 conjugated to keyhole limpet haemocyanin. The exact sequence is proprietary.
    Sequence:

    YLDDMVTCSQIVYNYNPEKTKKDSGWRTAICPDYCPNMYEEMETLASVLQ S


    Database link: Q7Z333

  • Positive control

    • IHC-P: Muscle of mouse embryo tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab214183 in the following tested applications.

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

Application Abreviews Notes
IHC-P 1/100 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Target

  • Function

    Probable helicase, which may be involved in RNA maturation (By similarity). Involved in DNA double-strand breaks damage response generated by oxidative stress.
  • Tissue specificity

    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).
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the DNA2/NAM7 helicase family.
  • Cellular localization

    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
  • Database links

  • Alternative names

    • 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

Images

  • Immunohistochemical analysis of formalin-fixed, paraffin-embedded muscle of mouse embryo tissue labeling Senataxin with ab214183 at 1/200 dilution, followed by secondary antibody detection and DAB staining.

References

ab214183 has not yet been referenced specifically in any publications.

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