| Gene Name |
ATXN1 |
| Synonyms |
ATXN1，ATX1，SCA1 |
| English Name |
Recombinant human ATXN1 protein |
| Full Name |
ataxin 1 |
| Appearance |
Powder: Lyophilized from a 0.2 μm filtered solution of 2-8M Urea, 20mM Tris-HCl, 150mM NaCl, 1mM DTT, PH7.2-8.0. |
| Purity |
≥85% by SDS-PAGE. |
| Storage |
Reconstituted protein solution can be stored at 4-7℃ for 1-2 weeks， stored at < -20℃ for 1 year. |
| Transport Condition |
The product is shipped at -20℃ temperature. Upon receipt, store it immediately at the temperature listed below. |
| Concentration |
1mg/ml by SDS-PAGE. |
| Swiss Prot |
P54253 |
| Gene ID |
6310 |
| Molecular Weight |
37kDa |
| Interval Range |
1-350aa |
| Background |
The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. ADCAI is genetically heterogeneous, with five genetic loci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6, being assigned to five different chromosomes. ADCAII, which always presents with retinal degeneration (SCA7), and ADCAIII often referred to as the `pure' cerebellar syndrome (SCA5), are most likely homogeneous disorders. Several SCA genes have been cloned and shown to contain CAG repeats in their coding regions. ADCA is caused by the expansion of the CAG repeats, producing an elongated polyglutamine tract in the corresponding protein. The expanded repeats are variable in size and unstable, usually increasing in size when transmitted to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6, and it has been determined that the diseased allele contains 40-83 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1). At least two transcript variants encoding the same protein have been found for this gene. |
| Application |
Immunology research |
| Source |
E.coli |
| Purification Method |
NI-NTA affinity purification |
| Solvent |
Reconstituted protein solution can be diluted with distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles. (It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water.) |
| Endotoxin |
Not measured |
| Biological Activity |
N0 |
| Lable Information |
With a 6×His tag at the N/C-terminus. |
| Unit |
Piece |
| Specification |
10ug 50ug 100ug 1mg |
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