Overview

  • Product name

    Anti-Nebulin antibody [Neb-20]
    See all Nebulin primary antibodies
  • Description

    Mouse monoclonal [Neb-20] to Nebulin
  • Host species

    Mouse
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Chicken, Human, Frog
  • Immunogen

    Chicken breast Nebulin.

Properties

Applications

Our Abpromise guarantee covers the use of ab130000 in the following tested applications.

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

Application Abreviews Notes
WB Use a concentration of 1 - 2 µg/ml. Predicted molecular weight: 773 kDa.

Target

  • 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
  • Database links

  • Alternative names

    • DKFZp686C1456 antibody
    • FLJ11505 antibody
    • FLJ36536 antibody
    • FLJ39568 antibody
    • FLJ39584 antibody
    • NEB 177D antibody
    • NEB antibody
    • NEB177D antibody
    • NEBU_HUMAN antibody
    • Nebulin antibody
    • NEM 2 antibody
    • NEM2 antibody
    see all

References

ab130000 has not yet been referenced specifically in any publications.

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