• Anti-ATXN3 antibody at 1:500 dilution; lysates at 100 µg per lane; rabbit polyclonal to goat IgG (HRP) at 1:10000 dilution

Anti-ATXN3 antibody (120aa to 250aa) (STJ140128)

SKU:
STJ140128

Current Stock:
Host: Goat
Applications: WB
Reactivity: Human/Rat/Mouse/Monkey/Canine
Note: STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.
Short Description: Goat polyclonal antibody anti-Ataxin 3 (120aa to 250aa) is suitable for use in Western Blot research applications.
Clonality: Polyclonal
Conjugation: Unconjugated
Isotype: IgG
Formulation: PBS. It does not contain preservatives. Glycerol and azide FREE.
Purification: This antibody is epitope-affinity purified from goat antiserum.
Concentration: 3 mg/ml
Dilution Range: WB 1:250-1:2000
Storage Instruction: For 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.
Gene Symbol: ATXN3
Gene ID: 4287
Uniprot ID: ATX3_HUMAN
Immunogen Region: 120aa to 250aa
Accession Number: ENSG00000066427
Specificity: Detects 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.
Immunogen: Purified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli.
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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