Anti-Ataxin 3 antibody [R04-1I8] (STJA0035121)
SPECIFICATIONS
ClonalityMonoclonal
HostRabbit
ConjugationUnconjugated
IsotypeIgG
ImmunogenA synthetic peptide of human Ataxin 3
General Information
| Short Description | Rabbit monoclonal anti-Ataxin 3 for use in WB in Human samples. Datasheet included with dilution recommendations, and related reagents. |
| Applications | WB |
| Host | Rabbit |
| Reactivity | Human |
| Note | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
Product Properties
| Clonality | Monoclonal |
| Clone ID | R04-1I8 |
| Isotype | IgG |
| Conjugation | Unconjugated |
| Concentration | 0.3 mg/mL |
| Purification | Affinity Purified |
| Dilution Range | WB 1:500-1:1000 |
| Formulation | 50mM Tris-Glycine (pH 7.4) , 0.15M NaCl, 40% Glycerol, 0.01% Sodium azide and 0.05% BSA |
| Storage Instruction | Store at 4°C short term. Aliquot and store at-20°C long term. Avoid freeze/thaw cycles. |
Target Information
| Gene Symbol | ATXN3 |
| Gene ID | 4287 |
| Uniprot ID | ATX3_HUMAN |
| Immunogen | A synthetic peptide of human Ataxin 3 |
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-3Machado-Joseph Disease Protein 1Spinocerebellar Ataxia Type 3 Protein |
| Database Links | Reactome: R-HSA-5689877Reactome: R-HSA-9615017 |
| Cellular Localisation | Nucleus MatrixNucleusLysosome MembranePeripheral Membrane ProteinPredominantly NuclearBut Not ExclusivelyInner Nuclear MatrixRecruited To Lysosomal Membrane In Response To Amino Acid Deprivation By The Raga/Rraga-Ragb/Rragb Complex |
| Alternative Antibody Names | Anti-Ataxin-3 antibodyAnti-Machado-Joseph Disease Protein 1 antibodyAnti-Spinocerebellar Ataxia Type 3 Protein antibodyAnti-ATXN3 antibodyAnti-ATX3 antibodyAnti-MJD antibodyAnti-MJD1 antibodyAnti-SCA3 antibody |
Information sourced from Uniprot.org