Myotubularin Polyclonal Antibody | MyBioSource MBS9412744 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Myotubularin Polyclonal Antibody

catalog :

MBS9412744

quantity :

0.05 mL

price :

200 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse

application :

western blot, ELISA, enzyme immunoassay

more info or order :

MyBioSource product webpage

image

image 1 :

Western Blot analysis of COLO205 cells using Myotubularin Polyclonal Antibody

product information

catalog number :

MBS9412744

products type :

Antibody

products full name :

Myotubularin Polyclonal Antibody

products short name :

[Myotubularin]

products name syn :

[MTM1; CG2; Myotubularin]

other names :

[myotubularin; Myotubularin; myotubularin; myotubularin 1; Phosphatidylinositol-3,5-bisphosphate 3-phosphatase (EC:3.1.3.95]

other gene names :

[MTM1; MTM1; CNM; MTMX; XLMTM; CG2]

uniprot entry name :

MTM1_HUMAN

clonality :

Polyclonal

host :

Rabbit

reactivity :

Human, Mouse

sequence length :

603

specificity :

Myotubularin Polyclonal Antibody detects endogenous levels of Myotubularin protein.

purity :

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

form :

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

concentration :

1 mg/ml

storage stability :

Store at -20°C/ 1 year

tested application :

Western Blot (WB), ELISA (EIA)

app notes :

Western Blot: 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.

image1 heading :

Western Blot (WB)

other info1 :

Immunogen Type: peptide. Immunogen Description: Synthesized peptide derived from the Internal region of human Myotubularin. SDS-PAGE MW: 70kD

other info2 :

Target Name: Myotubularin

products categories :

Total protein Ab

ncbi gi num :

4557896

ncbi acc num :

NP_000243.1

ncbi gb acc num :

NM_000252.2

uniprot acc num :

Q13496

ncbi pathways :

Inositol Phosphate Metabolism Pathway (82987); Inositol Phosphate Metabolism Pathway (362); Metabolic Pathways (132956); Metabolism Pathway (1269956); Metabolism Of Lipids And Lipoproteins Pathway (1270001); PI Metabolism Pathway (1270073); Phosphatidylinositol Signaling System Pathway (83052); Phosphatidylinositol Signaling System Pathway (461); Phospholipid Metabolism Pathway (1270053); Synthesis Of PIPs At The Early Endosome Membrane Pathway (1270079)

ncbi summary :

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]

uniprot summary :

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: Motility/polarity/chemotaxis; EC 3.1.3.64; EC 3.1.3.95; Protein phosphatase, dual-specificity. Chromosomal Location of Human Ortholog: Xq28. Cellular Component: ruffle; I band; late endosome; cytoplasm; plasma membrane; cytosol; filopodium. Molecular Function: phosphatidylinositol-3-phosphatase activity; protein binding; intermediate filament binding; protein tyrosine phosphatase activity; phosphoprotein phosphatase activity; phosphoinositide binding. Biological Process: muscle maintenance; protein transport; phospholipid metabolic process; phosphatidylinositol biosynthetic process; intermediate filament organization; endosome to lysosome transport; phosphoinositide dephosphorylation; protein amino acid dephosphorylation; mitochondrion distribution. Disease: Myopathy, Centronuclear, X-linked

size1 :

0.05 mL

price1 :

200 USD

size2 :

0.1 mL

price2 :

255

more info or order :

MyBioSource product webpage