产品名称 自分泌运动因子抗体
产品缩写 ATXN1
蛋 白 名 Ataxin-1
别 称 ATX1; SCA1; D6S504E
来 源 小鼠
特 异 性 抗人
分 子 量 87kDa
克隆类型 单克隆抗体：IgG1；克隆号：2F5
应 用 IH(P)免疫组化(石蜡)；IC细胞免疫化学；FC流式细胞检测；ELISA
成 分 Ascitic fluid containing 0.03% sodium azide.
免 疫 原 Purified recombinant fragment of human ATXN1 expressed in E. Coli.
保存条件 连续使用时4°C存储，保质期六个月；长期存储时建议分装为10ul以上小包装-20°C存储，并避免反复冻融，保质期一年。
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 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.Tissue specificity: Widely expressed throughout the body.
