HAP1 Pure 1/HAP1 50ug
货号：
611302 基本售价：
4305.0 元 规格：
50ug
产品信息
概述| 货号 | 611302 |
| 描述 | Huntingtons disease (HD) is an autosomal dominant neurodegenerative disorder caused by an expanding polyglutamine repeat in the IT15orhuntingtin gene. The mechanism(s) of pathogenesis are not known and the wide expression of Huntingtin protein does not explain the selective neuropathology of HD. HAP-1 (Huntingtin-associatedprotein1), identified by yeast two hybrid screening, interacts with the huntingtin protein. There are two isoforms of rat HAP1 (HAP1-A and HAP1-B) which differ in the length of their C-terminal regions. Both proteins are highly hydrophilic and their binding to the Huntingtin protein is enhanced by the expanded polyglutamine repeat. Human HAP1 shares 62% amino acid identity with HAP1-A. HAP1 is specifically expressed in the CNS where it is restricted to limbic structures, such as amygdala, hypothalamus, bed nucleus of the stria terminalis, and the septal nucleus. The subcellular association of HAP1 with microtubules and many types of membraneous organelles implicates it in vesicular transport. Thus, the specific neural interaction of HAP1 with Huntingtin protein may lead to abnormalities in vesicular transport that cause the neuropathology of HD. |
| 别名 | Huntingtin Associated Protein 1 |
| 同种亚型 | IgG1 |
| 反应种属 | Rat (QC Testing) Mouse (Tested in Development) |
| 来源宿主 | Mouse |
| 使用方法 | WB, IF |
性能| 供应商 | BD Pharmingen |
| 免疫原 | Rat HAP1-A aa. 100-289 |
| 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 |
