Overview

  • Product name

  • Description

    Rabbit polyclonal to MTM1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Mouse
    Predicted to work with: Rat, Horse, Chicken, Guinea pig, Cow, Cat, Dog, Human
  • Immunogen

    Synthetic peptide corresponding to a region within N terminal amino acids 5-54 (SASKYNSHSL ENESIKKVSQ DGVSQDVSET VPRLPGELLI TEKEVIYICP) of Mouse MTM1 (NP_001157664).

  • Positive control

    • Mouse thymus lysate

Properties

Applications

Our Abpromise guarantee covers the use of ab128318 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 70 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

Target

  • 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.
  • Involvement in 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.
  • Sequence similarities

    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.
  • Cellular localization

    Cytoplasm. 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
  • Database links

  • Alternative names

    • AF073996 antibody
    • CG2 antibody
    • CNM antibody
    • KIAA4176 antibody
    • mKIAA4176 antibody
    • Mtm antibody
    • Mtm1 antibody
    • MTM1_HUMAN antibody
    • MTMX antibody
    • Myotubular myopathy 1 antibody
    • Myotubularin antibody
    • XLMTM antibody
    see all

Images

  • Anti-MTM1 antibody (ab128318) at 1 µg/ml + Mouse thymus lysate at 10 µg

    Predicted band size: 70 kDa

References

ab128318 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Application
Western blot
Loading amount
10 µg
Gel Running Conditions
Reduced Denaturing (4-12% Bis tris)
Sample
Sheep Tissue lysate - whole (Heart, left ventricle)
Specification
Heart, left ventricle
Blocking step
Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: RT°C

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Verified customer

Submitted Apr 10 2015

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