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|Antigen||Myotubularin 1 (MTM1) Antibodies|
|Reactivity||Human, Mouse (Murine), Rat (Rattus) Alternatives|
|Conjugate||This MTM1 antibody is un-conjugated Alternatives|
Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Western Blotting (WB)
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Product Details anti-MTM1 AntibodyTarget Details MTM1 Application Details Handling Images
|Purification||Purified by Protein A.|
|Immunogen||KLH conjugated synthetic peptide derived from human MTM1/Myotubularin|
Target Details MTM1Product Details anti-MTM1 Antibody Application Details Handling Images back to top
|Alternative Name||MTM1 (MTM1 Antibody Abstract)|
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.
Synonyms: CG2, CNM, KIAA4176, mKIAA4176, Mtm, Mtm1, MTM1_HUMAN, MTMX, Myotubular myopathy 1, Myotubularin, XLMTM.
|Pathways||Inositol Metabolic Process, Skeletal Muscle Fiber Development|
Application DetailsProduct Details anti-MTM1 Antibody Target Details MTM1 Handling Images back to top
|Restrictions||For Research Use only|
HandlingProduct Details anti-MTM1 Antibody Target Details MTM1 Application Details Images back to top
|Buffer||Aqueous buffered solution containing 1 % BSA, 50 % glycerol and 0.09 % sodium azide.|
|Precaution of Use||This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.|
|Storage Comment||Store at -20°C|
|Expiry Date||12 months|
ImagesProduct Details anti-MTM1 Antibody Target Details MTM1 Application Details Handling back to top