Myotubularin Antibody

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Western Blot: Myotubularin Antibody [NBP1-51232] Extracts from COLO205 cells

Product Details

Summary
Product Discontinued
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Order Details


    • Catalog Number
      NBP1-51232
    • Availability
      Product Discontinued

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Myotubularin Antibody Summary

Immunogen
Synthetic peptide, corresponding to amino acids 256-3111 of Human Myotubularin.
Specificity
Myotubularin (Y290) pAb detects endogenous levels of Myotubularin protein.
Clonality
Polyclonal
Host
Rabbit
Gene
MTM1
Purity
Immunogen affinity purified
Innovator's Reward
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Applications/Dilutions

Dilutions
  • Western Blot 1:500-1:1000
Application Notes
Western blot (WB) analysis of Myotubularin (Y290) pAb in extracts from COLO205 cells
Theoretical MW
70 kDa.
Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.

Reactivity Notes

Cross reacts with Human and Mouse.

Packaging, Storage & Formulations

Storage
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
Buffer
PBS (approx. pH 7.2)
Preservative
0.05% Sodium Azide
Concentration
1.0 mg/ml
Purity
Immunogen affinity purified

Alternate Names for Myotubularin Antibody

  • CG2
  • CNM
  • EC 3.1.3.48
  • MTMX
  • myotubular myopathy 1
  • myotubularin 1
  • myotubularin
  • XLMTM

Background

X-linked recessive myotubular myopathy is a congenital muscular disease characterized by severe hypotonia and generalized muscle weakness that,in most cases, leads to early postnatal death. The gene responsible for myotubular myopathy MTM1 encodes a dual specificity phosphatase, named myotubularin, which is highly conserved through evolution. The gene for MTM1 is localized to a 300 kb critical region on human Xq128 between IDS and GRBRA3. Human MTM1, a 603 amino-acid protein, is mutated in myotubular myopathy. The largely related protein hMTMR2 is found mutated in a recessive form of Charcot-Marie-tooth neuropathy. Myotubularin is primarily a lipid phosphatase that acts on hosphatidylinositol 3-monophosphate and is involved in the regulation of the phosphatidylinositol 3-kinase (PI 3-kinase) pathway and membrane trafficking. Wildtype myotubularin can directly dephosphorylate PI 3-P and PI 4-P in vitro. Thus, it decreases PI 3-P levels by down-regulating PI 3-K activity and by facilitating the degradation of PI 3-P.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Bioinformatics

Gene Symbol MTM1