Product nameMTM1 overexpression 293T lysate (whole cell)
ab94167 is a 293T cell transfected lysate in which Human MTM1 has been transiently over-expressed using a pCMV-MTM1 plasmid. The lysate is provided in 1X Sample Buffer. Note: For more detailed how the transfected lysate was prepared view preparation notes
Tested applicationsSuitable for: WBmore details
Storage instructionsShipped on dry ice. Upon delivery aliquot and store at -20ºC. Avoid freeze / thaw cycles.
Storage bufferConstituents: 0.01% Bromophenol blue, 2.3% Beta mercaptoethanol, 2% Sodium lauryl sulfate, 0.788% Tris HCl, 10% Glycerol (glycerin, glycerine)
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BackgroundFunction: 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. Disease: Defects in MTM1 are the cause of centronuclear myopathy X-linked (XCNM) [MIM:310400]; also known as X-linked myotubular myopathy (XLMTM) or myotubular myopathy type 1 (MTM1). Centronuclear myopathies are congenital muscle disorders characterized by progressive muscular weakness and wasting involving mainly limb girdle, trunk, and neck muscles. It may also affect distal muscles. Weakness may be present during childhood or adolescence or may not become evident until the third decade of life. Ptosis is a frequent clinical feature. The most prominent histopathologic features include high frequency of centrally located nuclei in muscle fibers not secondary to regeneration, radial arrangement of sarcoplasmic strands around the central nuclei, and predominance and hypotrophy of type 1 fibers. Similarity: Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily. Contains 1 GRAM domain. Contains 1 myotubularin phosphatase domain. Domain: The GRAM domain mediates binding to PI(3,5)P2 and, with lower affinity, to other phosphoinositides.
Our Abpromise guarantee covers the use of ab94167 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use at an assay dependent dilution.|
ab94167 at 15μg/lane on an SDS-PAGE gel.
All lanes : Anti-MTM1 antibody (ab55532) at 1/500 dilution
Lane 1 : MTM1 293T Transfected Lysate - Positive Control (ab94167)
Lane 2 : 293T non-transfected lysate
Lysates/proteins at 25 µg per lane.
All lanes : Goat Anti-mouse IgG (H and L) HRP conjugate at 1/2500 dilution
ab94167 has not yet been referenced specifically in any publications.