Recombinant Human ATXN3 Protein, DDK-tagged
Cat.No. : | ATXN3-739H |
Product Overview : | Recombinant Human ATXN3 fused with DDK tag at C-terminal was expressed in Sf9 cells. |
- Specification
- Gene Information
- Related Products
Description : | Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. |
Source : | Sf9 cells |
Species : | Human |
Tag : | DDK |
Form : | 50mM Tris-HCl, pH8.0, 100mM glycine, 10% glycerol |
Molecular Mass : | 41.1 kDa |
Purity : | > 80% as determined by SDS-PAGE and Coomassie blue staining |
Concentration : | >50 ug/mL as determined by microplate BCA method |
Gene Name : | ATXN3 ataxin 3 [ Homo sapiens ] |
Official Symbol : | ATXN3 |
Synonyms : | ATXN3; ataxin 3; Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) , MJD, SCA3; ataxin-3; ATX3; JOS; josephin; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant ref; olivopontocerebellar ataxia 3; Machado-Joseph disease protein 1; spinocerebellar ataxia type 3 protein; Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3); AT3; MJD; MJD1; SCA3; |
Gene ID : | 4287 |
mRNA Refseq : | NM_001127696 |
Protein Refseq : | NP_001121168 |
MIM : | 607047 |
UniProt ID : | P54252 |
Products Types
◆ Recombinant Protein | ||
ATXN3-0367H | Recombinant Human ATXN3 Protein (E2-K361), Tag Free | +Inquiry |
Atxn3-478M | Recombinant Mouse Atxn3 Protein, MYC/DDK-tagged | +Inquiry |
ATXN3-3036H | Recombinant Human ATXN3 Protein, MYC/DDK-tagged | +Inquiry |
ATXN3-0368H | Recombinant Human ATXN3 Protein (E2-K361), His tagged | +Inquiry |
ATXN3-2786H | Recombinant Human ATXN3 protein(11-290 aa), C-His-tagged | +Inquiry |
◆ Lysates | ||
ATXN3-52HCL | Recombinant Human ATXN3 lysate | +Inquiry |
Related Gene
For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.
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Q&As (6)
Ask a questionThe clinical manifestations of SCA3 include ataxia (especially limb motor coordination disorder), myoclonus, ocular muscle movement abnormalities, extrapyramidal symptoms, dysphagia, language disorder, etc. As the disease progresses, patients have more and more significant difficulties with walking, balance, and other movements.
ATXN3 protein is involved in many important cellular processes, including protein degradation, cell signaling, apoptosis, RNA metabolism, etc. It plays an important role in the regulation of cellular functions.
At present, there is no cure for SCA3, but some adjuvant therapies can be used to improve symptoms. Physical therapy, speech therapy and swallowing function training can improve the quality of life of patients. In addition, there are some studies on protein degradation pathways for abnormal aggregation of ATXN3 protein, but they are still in the experimental stage.
In addition to its association with SCA3, ATXN3 protein may also be associated with some other diseases. For example, studies have shown that ATXN3 protein is related to the occurrence and development of neurodegenerative diseases such as Parkinson's disease and Huntington's disease, but the specific mechanism needs to be further studied and verified.
SCA3 is caused by mutations in the ATXN3 gene that lead to excessive repeat of polyglutamine (PolyQ) sequences in ATXN3 protein. This abnormal expansion will cause abnormal ATXN3 protein folding and aggregation, leading to impaired cell function and eventually triggering SCA3 symptoms.
ATXN3 protein is associated with autodominant genetic disease Spinocerebellar Ataxia Type 3 (SCA3). SCA3 is a neurodegenerative disease characterized by ataxia, extrapyramidal symptoms, and motor neuron dysfunction.
Customer Reviews (3)
Write a reviewATXN3 is very catalytic active and shows excellent catalytic performance even at low concentrations.
ATXN3 detection method is sensitive and can detect trace amounts of protein, which is well suited for our study.
Using ATXN3, the reproducibility of my experiments is very good, which facilitates subsequent data analysis and experimental manipulation.
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