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Monoclonal Anti-ATXN1 antibody produced in mouse
产品编号: | 3141480 |
规格: | clone 4C5, purified immunoglobulin, buffered aqueous solution |
包装规格: | 100 μG |
产品类别: | 进口试剂 |
品牌: | Sigma-Aldrich |
优惠价: | 立即咨询 |
产品别名
Monoclonal Anti-ATXN1 antibody produced in mouse
ATX1
D6S504E
SCA1
基本信息
NACRES | NA.41 |
General description【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) |
Immunogen【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 |
Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
产品性质
Quality Level【质量水平】 | 100 |
biological source【生物来源】 | mouse |
conjugate【偶联物】 | unconjugated |
antibody form【抗体形式】 | purified immunoglobulin |
antibody product type | primary antibodies |
clone【克隆】 | 4C5, monoclonal |
form【形式】 | buffered aqueous solution |
mol wt【分子量】 | antigen ~37.11 kDa |
species reactivity | human |
technique(s) | indirect ELISA: suitable western blot: 1-5 μg/mL |
isotype【同位素/亚型】 | IgG2bκ |
NCBI accession no.【NCBI登记号】 | NM_000332 |
UniProt accession no.【UniProt登记号】 | P54253 |
shipped in【运输】 | dry ice |
storage temp.【储存温度】 | −20℃ |
Gene Information | human ... ATXN1(6310) |
产品说明
Disclaimer【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. |
安全信息
Storage Class Code【储存分类代码】 | 13 - Non Combustible Solids |
WGK | WGK 1 |