| 克隆類型 ： |
Rabbit Polyclonal IgG |
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免 疫 原 ： |
Peptide derived from human Ataxin 1 C-terminus. |
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純化方式 ： |
Immunogen affinity purified |
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緩 沖 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
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產(chǎn)品描述 ： |
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 contains41-81 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. |
| 適用物種 ： |
Hu, Mo, Rat |
| 應(yīng)用說明 ： |
E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 別 名 ： |
ATX1; ATXN1; SCA1; Ataxin 1; Ataxin-1; Ataxin1; Spinocerebellar ataxia type 1; ATX1_HUMAN. |
| 保存條件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 標(biāo) 記 物 ： |
PLLABS公司可供應(yīng)生物素、辣根過氧化物酶、熒光素標(biāo)記抗體 |
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產(chǎn)品說明 ： |
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