Ataxin-2 Pure 22/Ataxin-2 50ug
货号：
611378 基本售价：
3726.0 元 规格：
-
产品信息
概述| 货号 | 611378 |
| 描述 | The hereditary ataxias are neurodegenerative disorders characterized by abnormalities of balance due to dysfunction of the cerebellum and cerebellar pathways. Spinocerebellar ataxias (SCAs) represent a heterogeneous group of disorders with a prevalence of about 1 in 10^5. Moderate expansion (36 or 37 units) of CAG repeats on the SCA2 gene leads to a gene product, ataxin-2, that has a long polyglutamine tract. Ataxin-2 is a basic protein with two domains (Sm1 and Sm2) that have been implicated in RNA splicing and protein interaction. Human ataxin-2 has significant homology with mouse ataxin-2 and ataxin-2 related protein. Ataxin-2 is expressed as early as day 8 of mouse embryogenesis and is detected in brain, heart, placenta, liver, skeletal muscle, and pancreas, but not in lung or kidney. Expression increases with age and the protein is localized to the cytoplasm in purkinje cells and other neuronal cell types. Thus, ataxin-2 is thought to be important for RNA splicing or protein complex formation in many normal tissues, while polyglutamine containing ataxin-2 leads to neuropathology. |
| 同种亚型 | IgG1 |
| 反应种属 | Human (QC Testing) Rat (Tested in Development) |
| 来源宿主 | Mouse |
| 使用方法 | WB, IF |
性能| 供应商 | BD Pharmingen |
| 免疫原 | Human Ataxin-2 aa. 713-904 |
| 存放说明 | The monoclonal antibody was purified from tissue culture supernatant or ascites by affinity chromatography.Store undiluted at -20°C.
|
| 存储溶液 | Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide. |
| 浓度 | 250 µg/ml |
