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.
Formulation
0.5mg/ml with 1% BSA and 0.01% sodium azide if reconstituted with 0.2ml sterile 1XPBS
Host
Mouse
Immunogen Region
Chicken breast nebulin was used as the immunogen for this Nebulin antibody.
Isotype
IgG1
Predicted Reactivity
Chicken
Reactivity
Chicken
Recombinant
No
Antigen
Nebulin
Gene Id
4703
Clone No
NB2
Format
Ascites
Purification
Ascites
Storage
After reconstitution, the Nebulin antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.