This gene encodes nebulin, a giant protein component of the cytoskeletal matrix that coexists with the thick and thin filaments within the sarcomeres of skeletal muscle.
In most vertebrates, nebulin accounts for 3 to 4% of the total myofibrillar protein.
The encoded protein contains approximately 30aa long modules that can be classified into 7 types and other repeated modules.
Protein isoform sizes vary from 600 to 800kD due to alternative splicing that is tissue-, species-, and developmental stage-specific.
Of the 183 exons in the nebulin gene, at least 43 are alternatively spliced, although exons 143 and 144 are not found in the same transcript.
Of the several thousand transcript variants predicted for nebulin, the RefSeq Project has decided to create three representative RefSeq records.
Mutations in this gene are associated with recessive nemaline myopathy.
Applications:Suitable for use in ELISA, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.3, 0.05% sodium azide, 50% glycerol.
Specificity：Recognizes human NEB.
Isotype：IgG
Calc Applications Abbrev：E IHC
Calc Crossreactivity：Hu
Immunogen：Synthetic peptide corresponding to human NEB.
