ARG56261
anti-Ataxin 3 antibody
anti-Ataxin 3 antibody for ICC/IF,IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human,Mouse,Rat
Overview
| Product Description | Rabbit Polyclonal antibody recognizes Ataxin 3 |
|---|---|
| Tested Reactivity | Hu, Ms, Rat |
| Tested Application | ICC/IF, IHC-P, WB |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | IgG |
| Target Name | Ataxin 3 |
| Antigen Species | Human |
| Immunogen | Recombinant protein of Human Ataxin-3 |
| Conjugation | Un-conjugated |
| Alternate Names | MJD; ATX3; JOS; Spinocerebellar ataxia type 3 protein; Ataxin-3; Machado-Joseph disease protein 1; AT3; SCA3; MJD1; EC 3.4.19.12 |
Application Instructions
| Application Suggestion |
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| Application Note | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||||||
| Positive Control | SW480 |
Properties
| Form | Liquid |
|---|---|
| Purification | Affinity purification with immunogen. |
| Buffer | PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. |
| Preservative | 0.02% Sodium azide |
| Stabilizer | 50% Glycerol |
| Storage Instruction | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
| Note | For laboratory research only, not for drug, diagnostic or other use. |
Bioinformation
| Database Links | |
|---|---|
| Gene Symbol | ATXN3 |
| Gene Full Name | ataxin 3 |
| Background | Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Sep 2009] |
| Function | Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription. [UniProt] |
| Calculated MW | 41 kDa |
| PTM | Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity. |
Images (3) Click the Picture to Zoom In
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ARG56261 anti-Ataxin 3 antibody ICC/IF image
Immunofluorescence: HeLa cells stained with ARG56261 anti-Ataxin 3 antibody.
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ARG56261 anti-Ataxin 3 antibody IHC-P image
Immunohistochemistry: Formalin/PFA-fixed paraffin-embedded Rat kidney tissue stained with ARG56261 anti-Ataxin 3 antibody at 1:200 dilution.
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ARG56261 anti-Ataxin 3 antibody WB image
Western blot: SW480 cell lysate stained with ARG56261 anti-Ataxin 3 antibody.
