Anti-ATXN3 antibody (190-361) [S4703RM] (STJ11104703)
SPECIFICATIONS
ClonalityMonoclonal
HostRabbit
ConjugationUnconjugated
IsotypeIgG
General Information
| Short Description | Rabbit monoclonal Ataxin-3 (190-361) antibody for use in WB and ELISA in mouse and rat samples. Datasheet included with dilution recommendations, and related reagents. |
| Applications | WB/ELISA |
| Host | Rabbit |
| Reactivity | Mouse/Rat |
| 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 | S4703RM |
| Isotype | IgG |
| Conjugation | Unconjugated |
| Concentration | Lot specific |
| Purification | Affinity purification |
| Dilution Range | WB:1:1000-1:5000ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Formulation | PBS with 0.05% Proclin300, 0.05% BSA, 50% Glycerol, pH 7.3. |
| Storage Instruction | Store at-20ยฐC for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
Target Information
| Gene Symbol | ATXN3 |
| Gene ID | 4287 |
| Uniprot ID | ATX3_HUMAN |
| Immunogen Region | 190-361 |
| Immunogen Sequence | KLIGEELAQLKEQRVHKTDL ERVLEANDGSGMLDEDEEDL QRALALSRQEIDMEDEEADL RRAIQLSMQGSSRNISQDMT QTSGTNLTSEELRKRREAYF EKQQQKQQQQQQQQQQGDLS GQSSHPCERPATSSGALGSD LGDAMSEEDMLQAAVTMSLE TVRNDLKTEGKK |
| Specificity | Recombinant fusion protein containing a sequence corresponding to amino acids 190-361 of human Ataxin-3 (ATXN3) (NP_004984.2). |
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