产品说明
一般描述
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 41-81 CAG repeats, compared to 6-39 in the normal allele. At least two transcript variants encoding the same protein have been found for this gene. (provided by RefSeq)
免疫原
ATXN1 (NP_000323, 576 a.a. ~ 675 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
KGSIIQLANGELKKVEDLKTEDFIQSAEISNDLKIDSSTVERIEDSHSPGVAVIQFAVGEHRAQVSVEVLVEYPFFVFGQGWSSCCPERTSQLFDLPCSK
外形
Solution in phosphate buffered saline, pH 7.4
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
| NACRES | NA.41 |
产品性质
| 质量水平 | 100 |
| 生物来源 | mouse |
| 偶联物 | unconjugated |
| 抗体形式 | purified immunoglobulin |
| antibody product type | primary antibodies |
| 克隆 | 4C5, monoclonal |
| 形式 | buffered aqueous solution |
| 分子量 | antigen ~37.11 kDa |
| species reactivity | human |
| technique(s) | indirect ELISA: suitable western blot: 1-5 μg/mL |
| 同位素/亚型 | IgG2bκ |
| NCBI登记号 | NM_000332 |
| UniProt登记号 | P54253 |
| 运输 | dry ice |
| 储存温度 | −20℃ |
| Gene Information | human ... ATXN1(6310) |
安全信息
| 储存分类代码 | 13 - Non Combustible Solids |
| WGK | WGK 1 |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
