Myotubularin (MTM1), ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of MTM1 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9356873 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Myotubularin (MTM1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing MTM1. The ELISA analytical biochemical technique of the MBS9356873 kit is based on MTM1 antibody-MTM1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MTM1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MTM1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

66,053 Da

myotubularin 1

myotubularin

Myotubularin

CG2  [Similar Products]

MTM1_HUMAN

This gene encodes a dual-specificity phosphatase that acts on both phosphotyrosine and phosphoserine. It is required for muscle cell differentiation and mutations in this gene have been identified as being responsible for X-linked myotubular myopathy. [provided by RefSeq, Jul 2008]

MTM1: 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. Defects in MTM1 are the cause of centronuclear myopathy X-linked (CNMX). 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. Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily.

Protein type: EC 3.1.3.95; Motility/polarity/chemotaxis; EC 3.1.3.64; Protein phosphatase, dual-specificity

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: cytoplasm; cytosol; filopodium; I band; late endosome; plasma membrane; ruffle

Molecular Function: intermediate filament binding; phosphatidylinositol-3-phosphatase activity; phosphoinositide binding; phosphoprotein phosphatase activity; protein binding; protein tyrosine phosphatase activity

Biological Process: endosome to lysosome transport; intermediate filament organization; mitochondrion distribution; muscle maintenance; phosphatidylinositol biosynthetic process; phosphoinositide dephosphorylation; phospholipid metabolic process; protein amino acid dephosphorylation; protein transport

Disease: Myopathy, Centronuclear, X-linked

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