Ataxin 3 antibody
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- Target See all Ataxin 3 (ATXN3) Antibodies
- Ataxin 3 (ATXN3)
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Reactivity
- Human
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Host
- Mouse
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Clonality
- Monoclonal
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Conjugate
- This Ataxin 3 antibody is un-conjugated
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Application
- Western Blotting (WB)
- Purification
- The antibody was purified by affinity chromatography.
- Clone
- 1H9-2
- Isotype
- IgG1 kappa
- Top Product
- Discover our top product ATXN3 Primary Antibody
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- Application Notes
- Optimal working dilution should be determined by the investigator.
- Restrictions
- For Research Use only
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- Concentration
- 0.5 mg/mL
- Buffer
- phosphate-buffered solution, pH 7.2, containing 0.05 % sodium azide.
- Preservative
- Sodium azide
- Precaution of Use
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
- Storage
- 4 °C
- Storage Comment
- Upon receipt, store undiluted between 2°C and 8°C.
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- Target
- Ataxin 3 (ATXN3)
- Alternative Name
- Ataxin-3 (ATXN3 Products)
- Synonyms
- Mjd antibody, zgc:56323 antibody, ATXN3 antibody, at3 antibody, jos antibody, mjd antibody, atx3 antibody, mjd1 antibody, sca3 antibody, ataxin-3 antibody, MJD1 antibody, MGC83584 antibody, AT3 antibody, ATX3 antibody, JOS antibody, MJD antibody, SCA3 antibody, 2210008M02Rik antibody, AI463012 antibody, AI647473 antibody, Sca3 antibody, Rsca3 antibody, ataxin 3 antibody, ataxin 3 L homeolog antibody, ataxin-3 antibody, atxn3 antibody, ATXN3 antibody, atxn3.L antibody, MICPUN_104046 antibody, MICPUCDRAFT_46658 antibody, Atxn3 antibody
- Background
- Ataxin-3 is an ubiquitously expressed protein which contains a poly-glutamine tract. Genetically abnormal expansion of the poly-glutamine repeats results in accumulation of ubiquitinated ataxin-3 protein aggregates and leads to a neurodegenerative disorder, Machado-Joseph disease (MJD). The protein has ubiquitin interacting motifs and a N-terminal Cysteine residue (C14) responsible for its deubiquitinating activity. Ataxin-3 binds and hydrolyzes polyubiquitinated proteins, and interacts with key proteasome components, such as p97/VCP and p45, indicating that Ataxin-3 may play functional roles in ubiquitin-proteasome system. The protein also act as a transcriptional repressor when interacting with transcriptional activators and coactivators.
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