Anti-ATXN3 antibody (120-250aa) (STJ140015)

SPECIFICATIONS
ClonalityPolyclonal
HostGoat
ConjugationUnconjugated
IsotypeIgG
ImmunogenPurified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli.
STJ140015
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General Information

Short DescriptionGoat polyclonal Ataxin 3 (120-250aa) antibody for use in WB in human, mouse, rat, bovine, canine, chicken, avian, donkey, feline, goat, guinea pig, hamster, horse, porcine, rabbit, sheep and simian samples. Datasheet included with dilution recommenda
ApplicationsWB
HostGoat
ReactivityHuman/Mouse/Rat/Bovine/Canine/Chicken/Avian/Donkey/Feline/Goat/Guinea Pig/Hamster/Horse/Porcine/Rabbit/Sheep/Simian
NoteSTRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.

Product Properties

ClonalityPolyclonal
IsotypeIgG
ConjugationUnconjugated
Concentration3 mg/mL
PurificationThis antibody is epitope-affinity purified from goat antiserum.
Dilution RangeWB 1:250-1:2000
FormulationPBS, 20% Glycerol and 0.05% Sodium Azide.
Storage InstructionFor continuous use, store at 2-8 C for one-two days. For extended storage, store in-20 C freezer. Working dilution samples should be discarded if not used within 12 hours.

Target Information

Gene SymbolATXN3
Gene ID4287
Uniprot IDATX3_HUMAN
Accession NumberENSG00000066427
ImmunogenPurified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli.
Immunogen Region120-250aa
Immunogen SequenceKEHWFTVRKLGKQWFNLNSL EHWFTVRKLGKQWFNLNSLL HWFTVRKLGKQWFNLNSLLT WFTVRKLGKQWFNLNSLLTG FTVRKLGKQWFNLNSLLTGP TVRKLGKQWFNLNSLLTGPE VRKLGKQWFNLNSLLTGPEL
SpecificityDetects levels of ATXN3 protein in transfected cells with GFP-ATXN3 and endogenous levels in U118, SH-SY5Y, At-T20 and hTERT cell lysates by Western blot.

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

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