ATXN3

Alternative Names: Ataxin-3, Spinocerebellar ataxia type 3 protein, Machado-Joseph disease protein 1, ATXN3, ATX3, MJD, MJD1, SCA3, AT3, JOS.

Ataxin 3, also called Machado-Joseph disease protein 1, is a neurodegenerative disorder caused by an expansion of trinucleotide CAG repeats in the ATXN3 gene. Normally, there are 13-36 repeats, but in Machado-Joseph disease, the repeats increase to 68-79. ATXN3 is a poly-ubiquitin-binding protein that is regulated by its catalytic activity. It is also involved in the degradation of ubiquitinated proteins as a proteasome-associated factor. ATXN3 can fold reversibly using a single intermediate, and when partially destabilized, it forms fibrillar aggregates in the non-pathological variant. It interacts with major histone acetyltransferases, including cAMP-response-element binding protein (CREB)-binding protein, p300, and p300/CREB-binding protein-associated factor, and inhibits their transcriptional coactivator function.

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  • Sku: PSB-pro-708-2µg
  • Vendor: ProSpec-Tany
$60.00 USD
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Safety Data Sheet
UniProt Listing
NCBI Link

Product Details

Product Description

Recombinant Human Ataxin-3 is a protein that belongs to the family of cysteine proteases. With a molecular weight of approximately 42 kDa, this protein plays a significant role in the pathogenesis of neurodegenerative disorders. It is involved in the ubiquitin-proteasome system, regulating protein degradation and maintaining cellular homeostasis. Recombinant Human Ataxin-3 is widely studied for its implications in diseases such as spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease, which is characterized by the expansion of a polyglutamine repeat within the ataxin-3 protein.

Current Lead Time

7-14 Days

Product Specifications

Species
 Human
Expression System E. coli
Purity 90% by SDS-PAGE
Activity
 Not Available
Activity Assay
Animal Component Free (ACF) Yes
Molecular Weight
 42.4 kDa
Structure Monomer
Endotoxin Concentration
 Not Available
Purification Method Not Available
Form
 Frozen
Formulation The ATXN3 protein solution contains 20mM Tris-HCl buffer (pH 7.5), 2mM DTT, 50mM NaCl and 10% glycerol.

*For Research Use Only

Product Guarantee

If your product is within its expiration period, and has not performed according to expectations, fill out the sample return form. Return the unused sample frozen, with the auto generated packing slip to the address on the instructions. You will receive a full refund along with a replacement of the unused portion (inclusive of the failed test). Damages do not cover any additional costs incurred for failed experimentation. 

Amino Acid Sequence

MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERMRMAE GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLELILFNSPEYQ RLRIDPINER SFICNYKEHW FTVRKLGKQW FNLNSLLTGP ELISDTYLAL FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQMHRPK LIGEELAQLK EQRVHKTDLE RVLEANDGSG MLDEDEEDLQ RALALSRQEI DMEDEEADLR RAIQLSMQGS SRNISQDMTQ TSGTNLTSEE LRKRREAYFE KQQQKQQQQQ QQQQQQQQQQ QQQQGDLSGQ SSHPCERPAT SSGALGSDLG DAMSEEDMLQ AAVTMSLETV RNDLKTEGKK.

Storage Instruction

Reconstituted store at -20°C or -80°C.We recommend that single use aliquots are prepared to avoid freeze-thaw cycles.

Sample Prep Instruction

1. Thaw the vial on ice, spin briefly
2. Prepare single-use or stock aliquots. This stage will depend on your final application so please adapt as appropriate. All our proteins are supplied carrier protein-free. If compatible with your work and you are storing at lower concentrations (<50 ug/ml) adding carrier protein is highly advised (usually 1% w/v high purity BSA or equivalent – ensure all buffers are sterile-filtered).
3. Prepare single-use aliquots whenever possible to avoid freeze-thaw cycles which can damage the proteins and reduce bioactivity. Store aliquots at -70 °C (or -20 °C)
4. We recommend sterile filtering after dilution in media or the final working solution
​

References

Not Available
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ATXN3
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