Ataxin 3 Antibody (YA9748)
(Synonyms: ATX3, MJD, MJD1, SCA3, ATXN3, Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein)
Based on 1 Customer Validation
Ataxin 3 Antibody (YA9748) is a Rabbit-derived and non-conjugated IgG Recombinant,Monoclonal antibody, targeting to Ataxin 3.
-
Host:
Rabbit
-
Isotype:
IgG
-
Application:
WB, ICC/IF, FC, IP
-
Reactivity :
Human
-
Formulation:
Supplied in PBS (pH7.4), 0.1% BSA, 40% Glycerol, 0.2% Proclin950.
-
Conjugation:
Non-conjugated
To place orders, for customer services and technical support, please contact: MedChemExpress USA
Tel: 609-228-6898 E-mail: [email protected] [email protected]
Applications
| Application |
WB
WB: Western Blot
|
ICC/IF
ICC/IF: Immunocytochemistry/
Immunofluorescence |
FC
FC: Flow Cytometry
|
IP
IP: Immunoprecipitation
|
|---|---|---|---|---|
| Dilution Ratio | 1:5000 | 1:100 | 1:1000 | 1-2 μg/sample |
Product Details
Ataxin 3 Antibody (YA9748) is a Rabbit-derived and non-conjugated IgG Recombinant,Monoclonal antibody, targeting to Ataxin 3.
-
Host Rabbit
-
Clonality Recombinant,Monoclonal
-
Species ReactivityHuman
-
Observed Molecular WeightObserved band size: 45 kDa
Note: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
-
Calculated Molecular Weight Predicted band size: 41 kDa
Recombinant protein within human Ataxin 3 (aa 1-361).
Endogenous
affinity purified.
Non-conjugated
Unmodified
IgG
Product Properties
-
Appearance
Solution
-
Formulation
Supplied in PBS (pH7.4), 0.1% BSA, 40% Glycerol, 0.2% Proclin950.
-
Concentration
Batch-dependent, Please check the COA for the concentration of each lot. Check Lot Concentration
-
Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
-
Shipping
Shipping with blue ice.
Background
-
Function
Ataxin 3 is a 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 (By similarity). 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[1][2][3][4][5][6].
-
Subcellular Localization
Nucleus matrix; Nucleus; Lysosome membrane
-
Expression
Tissue_Specificity: Ubiquitous. -
Isoforms & Post-Translational Modification
Ataxin 3 has 5 isoforms, P54252-2: amino acid length is 361, molecular weight is 41250 Da (predicted); P54252-1: amino acid length is 364, molecular weight is 41781 Da (predicted); P54252-3: amino acid length is 306, molecular weight is 35006 Da (predicted); P54252-4: amino acid length is 346, molecular weight is 39638 Da (predicted); P54252-5: amino acid length is 182, molecular weight is 20633 Da (predicted).Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity (PubMed:23696636)
-
Subunit
Interacts with STUB1/CHIP (when monoubiquitinated) (By similarity).
-
SwissProt ID
-
Synonyms
ATX3, MJD, MJD1, SCA3, ATXN3, Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein
Documentation
References
[1]. Li F, et al. Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities. J Biol Chem. 2002 Nov 22;277(47):45004-12. [Content Brief]
[2]. Mao Y, et al. Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain. Proc Natl Acad Sci U S A. 2005 Sep 6;102(36):12700-5. [Content Brief]
[3]. Tzvetkov N, et al. Josephin domain-containing proteins from a variety of species are active de-ubiquitination enzymes. Biol Chem. 2007 Sep;388(9):973-8. [Content Brief]
[4]. Seki T, et al. JosD1, a membrane-targeted deubiquitinating enzyme, is activated by ubiquitination and regulates membrane dynamics, cell motility, and endocytosis. J Biol Chem. 2013 Jun 14;288(24):17145-55. [Content Brief]
[5]. Ashkenazi A, et al. Polyglutamine tracts regulate beclin 1-dependent autophagy. Nature. 2017 May 4;545(7652):108-111. [Content Brief]
[6]. Yao Y, et al. Amino Acids Enhance Polyubiquitination of Rheb and Its Binding to mTORC1 by Blocking Lysosomal ATXN3 Deubiquitinase Activity. Mol Cell. 2020 Nov 5;80(3):437-451.e6. [Content Brief]
Powered by Bioz
