Telethonin Pure 53/Telethonin 50ug
货号：
612328 基本售价：
3726.0 元 规格：
50ug
产品信息
概述| 货号 | 612328 |
| 描述 | Autosomal recessive limb-girdle muscular dystrophy (AR LGMD), a genetically heterogeneous group of disorders affecting the proximal musculature, has eight distinct forms: 2A to 2H. Each of these forms is caused by specific gene mutations. A mutation in the telethonin gene causes LGMD 2G. Telethonin, a 19 kDa sarcomeric protein found in striated and cardiac muscle, has a developmentally and functionally regulated expression pattern. Telethonin transcript down-regulation occurs in response to muscle denervation. Protein levels of telethonin are partially regulated by neuronal activity, thereby linking telethonin to dynamic control of myofibrillogenesis and muscle turnover in human skeletal muscle. The telethonin transcript exhibits a pattern of accumulation typical of contractile proteins, suggesting a role for the protein in myofibrillar assembly. Immunofluorescence images show that telethonin co-localizes with myosin, which is an expression pattern typical of sarcomeric proteins. Telethonin has a known sarcomeric binding partner, titin, and is also known as titin CAP (TCAP). Therefore, telethonin plays a major role in AR LGMD 2G and is also important as a developmentally and functionally regulated sarcomeric protein. |
| 同种亚型 | IgG1 |
| 反应种属 | Mouse (QC Testing) Human, Rat (Tested in Development) |
| 来源宿主 | Mouse |
| 使用方法 | WB |
性能| 供应商 | BD Pharmingen |
| 免疫原 | Mouse Telethonin aa. 1-167 |
| 存放说明 | The monoclonal antibody was purified from tissue culture supernatant or ascites by affinity chromatography.Store undiluted at -20°C.
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| 存储溶液 | Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide. |
| 浓度 | 250 µg/ml |
