Anti-Senataxin antibody (ab220827)
Key features and details
- Rabbit polyclonal to Senataxin
- Suitable for: ICC/IF
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-Senataxin antibody
See all Senataxin primary antibodies -
Description
Rabbit polyclonal to Senataxin -
Host species
Rabbit -
Tested applications
Suitable for: ICC/IFmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Mouse, Rat -
Immunogen
Recombinant fragment corresponding to Human Senataxin aa 200-400.
Database link: Q7Z333 -
Positive control
- A549 cells.
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), 59% PBS -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab220827 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 |
---|---|---|
ICC/IF | (2) |
Use a concentration of 0.25 - 2 µg/ml.
Fixation/Permeabilization: PFA/Triton X-100. |
Notes |
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ICC/IF
Use a concentration of 0.25 - 2 µg/ml. Fixation/Permeabilization: PFA/Triton X-100. |
Target
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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
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Database links
- Entrez Gene: 23064 Human
- Entrez Gene: 269254 Mouse
- Entrez Gene: 362096 Rat
- Omim: 608465 Human
- SwissProt: Q7Z333 Human
- SwissProt: A2AKX3 Mouse
- Unigene: 460317 Human
- Unigene: 41867 Mouse
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Alternative names
- ALS4 antibody
- Amyotrophic lateral sclerosis 4 protein antibody
- AOA2 antibody
see all
Images
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (6)
ab220827 has been referenced in 6 publications.
- Kannan A et al. Mutation in senataxin alters the mechanism of R-loop resolution in amyotrophic lateral sclerosis 4. Brain 145:3072-3094 (2022). PubMed: 35045161
- Sims JR et al. Phosphoproteomics of ATR signaling in mouse testes. Elife 11:N/A (2022). PubMed: 35133275
- Kannan A et al. ZPR1 prevents R-loop accumulation, upregulates SMN2 expression and rescues spinal muscular atrophy. Brain 143:69-93 (2020). PubMed: 31828288
- Edwards DS et al. BRD4 Prevents R-Loop Formation and Transcription-Replication Conflicts by Ensuring Efficient Transcription Elongation. Cell Rep 32:108166 (2020). PubMed: 32966794
- Lam FC et al. BRD4 prevents the accumulation of R-loops and protects against transcription-replication collision events and DNA damage. Nat Commun 11:4083 (2020). PubMed: 32796829
- Kannan A et al. Combined deficiency of Senataxin and DNA-PKcs causes DNA damage accumulation and neurodegeneration in spinal muscular atrophy. Nucleic Acids Res 46:8326-8346 (2018). PubMed: 30010942