Key features and details
- Mouse monoclonal [Neb-20] to Nebulin
- Suitable for: WB
- Reacts with: Mouse, Rat, Chicken, Human, Frog
- Isotype: IgG1
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferPreservative: 0.001% Sodium azide
Constituents: 98% PBS, 0.2% BSA, 1.2% Sodium acetate
Concentration information loading...
PurityProtein G purified
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.
|WB||Use a concentration of 1 - 2 µg/ml. Predicted molecular weight: 773 kDa.|
FunctionThis 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 specificityMuscle specific. Located in the thin filament of striated muscle.
Involvement in diseaseDefects 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 similaritiesContains 178 nebulin repeats.
Contains 1 SH3 domain.
Cellular localizationCytoplasm > myofibril > sarcomere. Cytoplasm > cytoskeleton.
- Information by UniProt
- DKFZp686C1456 antibody
- FLJ11505 antibody
- FLJ36536 antibody
ab130000 has not yet been referenced specifically in any publications.