Anti-Ataxin 3 antibody [R01-1G-4] (STJA0033747)

SPECIFICATIONS
ClonalityMonoclonal
HostRabbit
ConjugationUnconjugated
IsotypeIgG
ImmunogenPeptide
STJA0033747
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General Information

Short DescriptionRabbit monoclonal anti-Ataxin 3 for use in IHC-P in Human, Mouse and Rat samples. Datasheet included with dilution recommendations, and related reagents.
ApplicationsIHC-P
HostRabbit
ReactivityHuman/Mouse/Rat
NoteSTRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.

Product Properties

ClonalityMonoclonal
Clone IDR01-1G-4
IsotypeIgG
ConjugationUnconjugated
PurificationProtein A
Dilution RangeIHC-P 1:50-1:100
FormulationLiquid in PBS, Glycerol and BSA
Storage InstructionStore at-20°C. Avoid freeze/thaw cycles.

Target Information

Gene SymbolATXN3
Gene ID4287
Uniprot IDATX3_HUMAN
ImmunogenPeptide

Additional Info

Tissue Specificity Ubiquitous.
Post Translational Modifications Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity.
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. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription. Acts as a negative regulator of mTORC1 signaling in response to amino acid deprivation by mediating deubiquitination of RHEB, thereby promoting RHEB inactivation by the TSC-TBC complex. Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1.
Protein Name Ataxin-3
Machado-Joseph Disease Protein 1
Spinocerebellar Ataxia Type 3 Protein
Database Links Reactome: R-HSA-5689877
Reactome: R-HSA-9615017
Cellular Localisation Nucleus Matrix
Nucleus
Lysosome Membrane
Peripheral Membrane Protein
Predominantly Nuclear
But Not Exclusively
Inner Nuclear Matrix
Recruited To Lysosomal Membrane In Response To Amino Acid Deprivation By The Raga/Rraga-Ragb/Rragb Complex
Alternative Antibody Names Anti-Ataxin-3 antibody
Anti-Machado-Joseph Disease Protein 1 antibody
Anti-Spinocerebellar Ataxia Type 3 Protein antibody
Anti-ATXN3 antibody
Anti-ATX3 antibody
Anti-MJD antibody
Anti-MJD1 antibody
Anti-SCA3 antibody

Information sourced from Uniprot.org

Citations

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