Sample:
Rectum (Mouse) Lysate at 40 ug
Primary: Anti- MTM1 (bs-9178R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 70 kD
Observed band size: 68 kD
- ￥1180 - 2800
- Bioss
- bs-9178R
- 2025年10月24日
- 产品信息以Bioss网站为准
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- 规格：
50ul/100ul/200ul
| 规格： | 50ul | 产品价格： | ￥1180.0 |
|---|---|---|---|
| 规格： | 100ul | 产品价格： | ￥1980.0 |
| 规格： | 200ul | 产品价格： | ￥2800.0 |
| 产品编号 | bs-9178R |
| 英文名称 | MTM1 Rabbit pAb |
| 中文名称 | 肌微管素1抗体 |
| 英文别名 | CG2; CNM; KIAA4176; mKIAA4176; Mtm; Mtm1; MTM1_HUMAN; MTMX; Myotubular myopathy 1; Myotubularin; XLMTM. |
| 产品应用 | WB=1:500-2000 Not yet tested in other applications. |
| 交叉反应 | Mouse (Human, Rat, Dog, Pig, Rabbit) |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human MTM1/Myotubularin |
| 亚型 | IgG |
| 性状 | Liquid |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 理论分子量 | 70 kDa |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Cell Biology > Cell Cycle > Cell differentiation Signal Transduction > Metabolism > Lipid metabolism Signal Transduction > Protein Phosphorylation > Ser / Thr Phosphatases Signal Transduction > Protein Phosphorylation > Tyrosine Phosphatases Stem Cells > Mesenchymal Stem Cells > Myogenesis |
| 亚基 | Interacts with MTMR12; the interaction modulates MTM1 intracellular localization. Interacts with MLL (via SET domain). Interacts with DES in skeletal muscle but not in cardiac muscle. |
| 亚细胞定位 | Cytoplasm. Cell membrane; Peripheral membrane protein. Cell projection, filopodium. Cell projection, ruffle. Late endosome. Note=Localizes as a dense cytoplasmic network. Also localizes to the plasma membrane, including plasma membrane extensions such as filopodia and ruffles. Predominantly located in the cytoplasm following interaction with MTMR12. Recruited to the late endosome following EGF stimulation. [DOMAIN] The GRAM domain mediates binding to PI(3,5)P2 and, with lower affinity, to other phosphoinositides. |
| 相似性 | Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily. Contains 1 GRAM domain. Contains 1 myotubularin phosphatase domain. |
| 功能 | Lipid phosphatase which dephosphorylates phosphatidylinositol 3-monophosphate (PI3P) and phosphatidylinositol 3,5-bisphosphate (PI(3,5)P2). Has also been shown to dephosphorylate phosphotyrosine- and phosphoserine-containing peptides. Negatively regulates EGFR degradation through regulation of EGFR trafficking from the late endosome to the lysosome. Plays a role in vacuolar formation and morphology. Regulates desmin intermediate filament assembly and architecture. Plays a role in mitochondrial morphology and positioning. Required for skeletal muscle maintenance but not for myogenesis. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | X-linked recessive myotubular myopathy is a congenital muscular disease characterized by severe hypotonia and generalized muscle weakness that, in most cases, leads to early postnatal death. The gene responsible for myotubular myopathy MTM1 encodes a dual specificity phosphatase, named myotubularin, which is highly conserved through evolution. The gene for MTM1 is localized to a 300 kb critical region on human Xq128 between IDS and GRBRA3. Human MTM1, a 603 amino-acid protein, is mutated in myotubular myopathy. The largely related protein hMTMR2 is found mutated in a recessive form of Charcot-Marie-Tooth neuropathy. Myotubularin is primarily a lipid phosphatase that acts on phosphatidylinositol 3-monophosphate and is involved in the regulation of the phosphatidylinositol 3-kinase (PI3-kinase) pathway and membrane trafficking. Wild-type myotubularin can directly dephosphorylate PI3P and PI4P in vitro. Thus, it decreases PI3P levels by down-regulating PI3K activity and by facilitating the degradation of PI3P. |
| 应用 | 推荐稀释比例 |
| {WB} | {1:500-2000} |
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MTM1 Rabbit pAb(bs-9178R)-50ul/100ul/200ul
￥1180 - 2800
