ARG43171
anti-Dysferlin antibody
anti-Dysferlin antibody for ICC/IF,IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human,Mouse
概述
| 产品描述 | Rabbit Polyclonal antibody recognizes Dysferlin |
|---|---|
| 反应物种 | Hu, Ms |
| 应用 | ICC/IF, IHC-P, WB |
| 宿主 | Rabbit |
| 克隆 | Polyclonal |
| 同位型 | IgG |
| 靶点名称 | Dysferlin |
| 抗原物种 | Human |
| 抗原 | Synthetic peptide derived from Human Dysferlin. |
| 偶联标记 | Un-conjugated |
| 別名 | FER1L1; Dystrophy-associated fer-1-like protein; Fer-1-like protein 1; LGMD2B; MMD1; Dysferlin |
应用说明
| 应用建议 |
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|---|---|---|---|---|---|---|---|---|---|
| 应用说明 | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||||||
| 阳性对照 | Mouse skeletal muscle | ||||||||
| 实际分子量 | ~ 270 kDa |
属性
| 形式 | Liquid |
|---|---|
| 纯化 | Affinity purified. |
| 缓冲液 | PBS (pH 7.4), 150 mM NaCl, 0.02% Sodium azide and 50% Glycerol. |
| 抗菌剂 | 0.02% Sodium azide |
| 稳定剂 | 50% Glycerol |
| 存放说明 | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
| 注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
| 数据库连接 | |
|---|---|
| 基因名称 | DYSF |
| 全名 | dysferlin |
| 背景介绍 | The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008] |
| 生物功能 | Key calcium ion sensor involved in the Ca(2+)-triggered synaptic vesicle-plasma membrane fusion. Plays a role in the sarcolemma repair mechanism of both skeletal muscle and cardiomyocytes that permits rapid resealing of membranes disrupted by mechanical stress (By similarity). [UniProt] |
| 细胞定位 | Cell membrane, sarcolemma. Cytoplasmic vesicle membrane. Cell membrane. Note=Colocalizes, during muscle differentiation, with BIN1 in the T-tubule system of myotubules and at the site of contact between two myotubes or a myoblast and a myotube. Wounding of myotubes led to its focal enrichment to the site of injury and to its relocalization in a Ca(2+)-dependent manner toward the plasma membrane. Colocalizes with AHNAK, AHNAK2 and PARVB at the sarcolemma of skeletal muscle. [UniProt] |
| 预测分子量 | 237 kDa |
检测图片 (1) Click the Picture to Zoom In
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ARG43171 anti-Dysferlin antibody WB image
Western blot: Mouse skeletal muscle lysate stained with ARG43171 anti-Dysferlin antibody.
