Ataxin 3 Antibody (YA1294)
(Synonyms: ATX3, MJD, MJD1, SCA3, ATXN3)Ataxin 3 Antibody (YA1294) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to Ataxin 3.
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Host:
Rabbit
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Isotype:
IgG
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Application:
IHC-P
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Reactivity :
Human, Mouse, Rat
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Formulation:
Supplied in PBS, Glycerol and BSA
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Conjugation:
Non-conjugated
Applications
| Application |
IHC-P-1
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|---|---|
| Dilution Ratio | 50-1:100 |
Product Details
Ataxin 3 Antibody (YA1294) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to Ataxin 3.
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Host Rabbit
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Clonality Recombinant,Monoclonal
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Species ReactivityHuman, Mouse, Rat
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Observed Molecular WeightObserved band size: 41 kDaNote: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
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Calculated Molecular Weight Predicted band size: 41 kDa
Entrez Gene: 4287 Human ; 110616 Mouse ; 60331 Rat
SwissProt: P54252 Human ; Q9CVD2 Mouse ; O35815 Rat
OMIM: 109150 Human
Peptide
Endogenous
Protein A
Non-conjugated
Unmodified
IgG
Product Properties
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Appearance
Liquid
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Formulation
Supplied in PBS, Glycerol and BSA
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Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
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Shipping
Shipping with blue ice.
Background
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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. 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].
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Subcellular Localization
Nucleus matrix; Nucleus; Lysosome membrane; Peripheral membrane protein
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Expression
Tissue_specificity:Ubiquitous -
Isoforms & Post-Translational Modification
P54252 has 5 isomers: P54252-2: 41250 Da (predicted); P54252-1: 41781 Da (predicted); P54252-3: 35006 Da (predicted); P54252-4: 39638 Da (predicted); P54252-5: 20633 Da (predicted).
Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity (PubMed:23696636) -
Subunit
Interacts with DNA repair proteins RAD23A and RAD23B (PubMed:16020535, PubMed:30455355). Interacts with BECN1 (via its poly-Gln domain) (PubMed:28445460). Interacts with PRKN, UBR2, VCP and tubulin. Short isoform 1 interacts with CASP7 (PubMed:30455355)
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SwissProt ID
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Synonyms
ATX3, MJD, MJD1, SCA3, ATXN3
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Research Field
Neuroscience
Documentation