Nebulin (NEB) Rabbit Polyclonal Antibody
Product Data | |
Application | IHC |
---|---|
Recommended Dilution | IHC: 50-200 Positive control: Human lung cancer Predicted cell location: Cytoplasm |
Reactivity | Human |
Antibody Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | Synthetic peptide corresponding to a region derived from 118-132 amino acids of human nebulin |
Buffer | PBS pH7.3, 0.05% NaN3, 50% glycerol |
Concentration | lot specific |
Purification | Antigen affinity purification |
Conjugation | Unconjugated |
Storage | Store at -20°C as received. |
Stability | Stable for 12 months from date of receipt. |
Shipping | Blue Ice |
Gene Name | nebulin |
Database Link | |
Background | 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 30-amino acid long modules that can be classified into 7 types and other repeated modules. Protein isoform sizes vary from 600 to 800 kD 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. |
Synonyms | NEB177D; NEM2 |
Reference Data | |
Protein Families | Druggable Genome |
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