Anti-ATXN1 Polyclonal Antibody-一抗-抗体-蛋白与免疫

Anti-ATXN1 Polyclonal Antibody

品牌:solarbio | 货号:K004371P

英文名称 Anti-ATXN1 Polyclonal Antibody
宿主 Rabbit
别名 ATX1;D6S504E;SCA1
应用 WB IF
稀释比例 WB 1:500-2000. IF 1:10-100.
交叉反应 Human Mouse Rat
蛋白分子量 87kDa
Gene ID 6310
保存 Store at -20°C. Avoid freeze / thaw cycles.
储存液 Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
纯化方法 Affinity purification
亚型 IgG
免疫原 Recombinant protein of human ATXN1
性状 液体
Public Immunogen Range Recombinant protein of human ATXN1
Subcellular Locations Cytoplasm Nucleus
Swiss Prot P54253
克隆类型 Polyclonal Antibody
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.