中文 
别名:ATX1; SCA1; D6S504E应用:WB
反应种属:Human, Mouse, Rat
规格：50μl/100μl
| Description |
|---|
| 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). Alternative splicing results in multiple transcript variants, with one variant encoding multiple distinct proteins, ATXN1 and Alt-ATXN1, due to the use of overlapping alternate reading frames. |
| Specification | |
|---|---|
| Aliases | ATX1; SCA1; D6S504E |
| Swissprot | P54253 |
| WB Predicted band size | 87 kDa |
| Host/Isotype | Rabbit IgG |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | Fusion protein of human ATXN1 |
| Formulation | pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
| Application | |
|---|---|
| WB | 1/500-1/2000 |
| ELISA | 1/5000-1/10000 |
 |
Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-6: HT29, A549, PC3, A375, Hepg2, Hela cell lysates, Primary antibody: P13868(ATXN1 Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 10 seconds |
本公司的所有产品仅用于科学研究或者工业应用等非医疗目的，不可用于人类或动物的临床诊断或治疗，非药用，非食用。
暂无评论
本公司的所有产品仅用于科学研究或者工业应用等非医疗目的，不可用于人类或动物的临床诊断或治疗，非药用，非食用。
发表回复