Description
Background
Nebulin is 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 and its size varies from 600 to 800 kD in a manner that is tissue-, species-, and developmental stage-specific. The nebulin gene contains 183 exons in a 249-kb genomic region. Nebulin is mapped to chromosome 2. Nebulin is a giant filamentous protein specific for vertebrate skeletal muscles. Mutations in the nebulin gene associated with autosomal recessive nemaline myopathy.
Data Sheet
| Application | Cross ReactivityHuman, mouse, rat, chicken, frog. |
| Clone | Neb-20 |
| Cross Reactivity | Human, mouse, rat, chicken, frog. |
| Form | lyophilized |
| Ig type | mouse IgG1 |
| Immunogen/Antigen | Chicken breast nebulin. |
| Reconstitution | 1.2% sodium acetate or neutral PBS. If 1ml of PBS is used, |
| Size | 100ug/vial |
| Storage | At -20C for one year. After reconstitution, at 4C for one |
