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折扣/货期| 货号：GTX11434 | 规格：50μg | 目录价：￥4700 |
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 7 antibody
别名:
ataxin 7 , ADCAII , OPCA3 , SCA7 , SGF73
反应种属:
Drosophila, Human, Mouse, Rat
宿主来源:
Rabbit
实验应用:
FCM, ICC/IF, IHC, IHC (Free Floating), IHC-Fr, WB
同种型:
IgG
免疫原:
Synthetic Peptide: M(1) S E R A A D D V R G E P R R A A(17) C
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by antigen-affinity chromatography
偶联:
Unconjugated
产品浓度:
1 mg/ml (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
预期分子量:
95
产品形式:
Liquid
存储溶液:
PBS, 0.1% BSA, 0.05% 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. This locus has been mapped to chromosome 3, and it has been determined that the diseased allele associated with spinocerebellar ataxia-7 contains 37-306 CAG repeats (near the N-terminus), compared to 4-35 in the normal allele. The encoded protein is a component of the SPT3/TAF9/GCN5 acetyltransferase (STAGA) and TBP-free TAF-containing (TFTC) chromatin remodeling complexes, and it thus plays a role in transcriptional regulation. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2016]
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