Ataxin 1 antibody-多抗-深圳欣博盛生物科技有限公司

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Ataxin 1 antibody

品牌：GeneTex

官网链接

说明书

折扣/货期

货号：GTX87190

规格：100μg

目录价：￥5300

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

别名:

ATX1 , ATXN1 , D6S504E , SCA1 , ataxin 1 , Ataxin 1

反应种属:

Human, Mouse

宿主来源:

Rabbit

实验应用:

ICC/IF, IHC-P

同种型:

IgG

免疫原:

The antiserum was produced against synthesized peptide derived from human Ataxin 1 (742-791).

克隆性:

Polyclonal

克隆号:

纯化方式:

Purified by antigen-affinity chromatography

偶联:

Unconjugated

产品浓度:

Batch dependent (Please refer to the vial label for the specific concentration.)

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, 150mM NaCl, 50% Glycerol, 0.02% Sodium azide.

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 41-81 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. [provided by RefSeq, Jan 2010]

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