靶标 ATXN1 别名 ATXN1;ATX1;D6S504E;SCA1;ataxin-1 宿主 Rabbit 稀释比例 IHC 1:50-200. Gene ID 6310 保存 Store at -20°C. Avoid freeze / thaw cycles. Swiss Prot P54253 蛋白分子量 87kDa 储存液 Buffer: PBS with 0.03% Proclin300, 50% glycerol, pH7.3. 亚细胞定位 Cytoplasm Cell membrane Nucleus 免疫原 A synthetic peptide of human ATXN1 亚型 IgG 纯化方法 Affinity purification 背景资料 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). At least two transcript variants encoding the same protein have been found for this gene. Public Immunogen Range 695-745/815aa 克隆类型 Polyclonal Antibody 交叉反应 Human Rat Mouse 应用 IHC 规格 50ul 100ul 使用前请离心。如有问题请及时联系我们。