Recombinant Human Nebulin protein (ab114753)

Overview

  • Product name
    Recombinant Human Nebulin protein
  • Protein length
    Protein fragment

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      NTKTSYHTPADMLSVTAAKDAQANITNTNYKHLIHKYILLPDAMNIELTR NMNRIQSDNEYKQDYNEWYKGLGWSPAGSLEVEKAKKATEYASDQKYRQH PSNFQFKKLT
    • Molecular weight
      38 kDa including tags
    • Amino acids
      2108 to 2217

Specifications

Our Abpromise guarantee covers the use of ab114753 in the following tested applications.

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

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 µg/ul.
    Best use within three months from the date of receipt of this protein.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • DKFZp686C1456
    • FLJ11505
    • FLJ36536
    • FLJ39568
    • FLJ39584
    • NEB
    • NEB 177D
    • NEB177D
    • NEBU_HUMAN
    • Nebulin
    • NEM 2
    • NEM2
    see all
  • Function
    This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Bind and stabilize F-actin.
  • Tissue specificity
    Muscle specific. Located in the thin filament of striated muscle.
  • Involvement in disease
    Defects in NEB are the cause of nemaline myopathy type 2 (NEM2) [MIM:256030]. A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination.
  • Sequence similarities
    Contains 178 nebulin repeats.
    Contains 1 SH3 domain.
  • Cellular localization
    Cytoplasm > myofibril > sarcomere. Cytoplasm > cytoskeleton.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE Stained with Coomassie Blue showing ab114753 at approximately 37.73kDa.

References

ab114753 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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