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折扣/货期| 货号：GTX642154 | 规格：100μl | 目录价：￥4000 |
| 货号：GTX642154-S | 规格：25μl | 目录价：￥1700 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
Ataxin 1 antibody [GT106]
别名:
ataxin 1 , ATX1 , D6S504E , SCA1
反应种属:
Mouse, Rat
宿主来源:
Mouse
实验应用:
IHC-P, WB
靶标/特异性:
Highly recommended for IHC-P in mouse tissues.
同种型:
IgG2b
免疫原:
The immunogen used to generate this antibody corresponds to human Ataxin 1.
克隆性:
Monoclonal
克隆号:
GT106
纯化方式:
Affinity purified by Protein A.
偶联:
Unconjugated
产品浓度:
1 mg/ml
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
产品形式:
Liquid
存储溶液:
PBS, no preservatives.
生产商:
GeneTex
功能与背景:
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). Alternative splicing results in multiple transcript variants, with one variant encoding multiple distinct proteins, ATXN1 and Alt-ATXN1, due to the use of overlapping alternate reading frames. [provided by RefSeq, Nov 2017]
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