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Anti-MTM1 Polyclonal Antibody is a Rabbit antibody targeting MTM1. Anti-MTM1 Polyclonal Antibody can be used in WB.
| Pack Size | Price | USA Warehouse | Global Warehouse | Quantity |
|---|---|---|---|---|
| 50 μL | $220 | 7-10 days | 7-10 days | |
| 100 μL | $371 | 7-10 days | 7-10 days | |
| 200 μL | $529 | 7-10 days | 7-10 days |
| Description | Anti-MTM1 Polyclonal Antibody is a Rabbit antibody targeting MTM1. Anti-MTM1 Polyclonal Antibody can be used in WB. |
| Ig Type | IgG |
| Reactivity | Mouse (predicted:Human,Rat,Dog,Pig,Rabbit) |
| Application | |
| Recommended Dose | WB: 1:500-2000 |
| Antibody Type | Polyclonal |
| Host Species | Rabbit |
| Subcellular Localization | 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. |
| Construction | Polyclonal Antibody |
| Purification | Protein A purified |
| Appearance | Liquid |
| Formulation | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Concentration | 1mg/ml |
| Research Background | 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. |
| Immunogen | KLH conjugated synthetic peptide: human MTM1/Myotubularin |
| Antigen Species | Human |
| Gene Name | MTM1 |
| Gene ID | |
| Protein Name | Myotubularin |
| Uniprot ID | |
| Function | 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. |
| Molecular Weight | Theoretical: 70 kDa. |
| Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| Transport | Shipping with blue ice. |
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