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Rabbit Anti-MTM1 Antibody (CSB-PA614418ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Phosphatidylinositol-3 Antibody,5-bisphosphate 3-phosphatase Antibody,Phosphatidylinositol-3-phosphate phosphatase Antibody,MTM1 Antibody,CG2 AntibodyMore alternative names for the antibody
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
Anti-MTM1 antibody (ab103626)
Anti-MTM1 antibody (ab103626)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Myotubularin protein (484-603AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Protein 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|| |
Myopathy, centronuclear, X-linked (CNMX) [MIM:310400]: A congenital muscle disorder 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. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily. The GRAM domain mediates binding to PI(3,5)P2 and, with lower affinity, to other phosphoinositides.
|Protein cellular 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. .
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CUSABIO BIOTECH CO.
|Product type|| |
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