Analytical Data
-
Gene name
Ataxin-3
-
简介
Ataxin-3 is a deubiquitinating enzyme that maintains proteostasis, regulates transcription, and is involved in cytoskeletal dynamics, myogenesis, and degradation of misfolded chaperone substrates. It trims long polyubiquitin chains and interacts with STUB1/CHIP to limit ubiquitin chain length, preventing further elongation. Ataxin-3 Protein, Human (His) is the recombinant human-derived Ataxin-3 protein, expressed by E. coli , with N-6*His labeled tag.
- Application
-
Biological Activity
Measured in a cell proliferation assay using SH-SY5Y human neuroblastoma cells. The ED50 this effect is 2.979 ng/ml, corresponding to a specific activity is 3.36×105 units/mg. Measured in a cell proliferation assay using SH-SY5Y human neuroblastoma cells. The ED50 for this effect is 2.979 ng/ml, corresponding to a specific activity is 3.36×105 units/mg.
-
Alternative Names
Ataxin-3; ATXN3; Machado-Joseph disease protein 1; Spinocerebellar ataxia type 3 protein; ATX3, MJD, MJD1; SCA3
-
Species
Human
-
Source
E. coli
-
Tag
N-6*His
-
Purity
Greater than 95% as determined by SDS-PAGE.
-
Uniprot
P54252-2
-
Expression Region
M1-K361
-
AA Sequence
MESIFHEKQEGSLCAQHCLNNLLQGEYFSPVELSSIAHQLDEEERMRMAEGGVTSEDYRTFLQQPSGNMDDSGFFSIQVISNALKVWGLELILFNSPEYQRLRIDPINERSFICNYKEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLKEQRVHKTDLERVLEANDGSGMLDEDEEDLQRALALSRQEIDMEDEEADLRRAIQLSMQGSSRNISQDMTQTSGTNLTSEELRKRREAYFEKQQQKQQQQQQQQQQGDLSGQSSHPCERPATSSGALGSDLGDAMSEEDMLQAAVTMSLETVRNDLKTEGKK
-
Protein Length
Full Length of Isoform-2
-
Molecular Weight
43 kDa
-
Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
-
Form
Freeze-dried powder
-
Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
-
Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
-
Stability Test
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
-
Storage & Shelf Life
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
-
Shipping
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
Related Products
Protein Description
Ataxin-3 is a polyglutamine (polyQ) protein associated with Machado-Joseph disease (MJD), a neurodegenerative disorder characterized by motor dysfunction, ataxia, and cognitive decline. The gene responsible for MJD, ATXN3, encodes the ataxin-3 protein, which contains a variable number of glutamine repeats. The pathogenesis of MJD is linked to the expansion of these repeats, leading to the misfolding and accumulation of ataxin-3, which disrupts cellular functions. The study of recombinant ataxin-3 proteins has become essential for understanding the molecular mechanisms underlying MJD. These recombinant proteins allow researchers to investigate the structure-function relationships of ataxin-3, particularly how polyQ expansion affects its interactions with other cellular partners and its role in cellular processes such as ubiquitin-proteasome system function and autophagy. Additionally, recombinant ataxin-3 is used to develop potential therapeutic strategies, including small molecules and gene therapy approaches, aimed at modulating its aggregation and restoring normal cellular functions. Overall, this research offers crucial insights into the disease mechanisms and potential avenues for intervention in MJD and related disorders.
