The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notesIF: 1/400.
WB: Use at an assay dependent dilution. Detects a band of approximately 500 kDa.
By indirect immunofluorescence, the antibody shows a typical striated staining pattern of frozen sections of chicken skeletal muscle. It stains within the I-band and shows a decoration line at a distance of 0.22 µm from the z-line in electron micrographs.
Not tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
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.