Key features and details
- Rabbit polyclonal to MTM1
- Suitable for: IHC-P, ICC/IF
- Reacts with: Human
- Isotype: IgG
Product nameAnti-MTM1 antibody
DescriptionRabbit polyclonal to MTM1
Tested applicationsSuitable for: IHC-P, ICC/IFmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Cow, Orangutan
- IHC-P: Human kidney tissue. ICC/IF: U-251 MG cells.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), PBS
Concentration information loading...
Our Abpromise guarantee covers the use of ab223719 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
|ICC/IF||Use a concentration of 0.25 - 2 µg/ml.
Fixation/Permeabilization: PFA/Triton X-100.
FunctionLipid 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.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily.
Contains 1 GRAM domain.
Contains 1 myotubularin phosphatase domain.
DomainThe GRAM domain mediates binding to PI(3,5)P2 and, with lower affinity, to other phosphoinositides.
Cellular localizationCytoplasm. Cell membrane. Cell projection > filopodium. Cell projection > ruffle. Late endosome. 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.
- Information by UniProt
- AF073996 antibody
- CG2 antibody
- CNM antibody
ab223719 has not yet been referenced specifically in any publications.