Nebulin Rabbit anti-Human, Polyclonal, Bioss
Rabbit Polyclonal Antibody
Manufacturer: Bioss BS2401R
DescriptionThis 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 kDa 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. Mutations in this gene are associated with recessive nemaline myopathy.
|Immunofluorescence, Immunohistochemistry (Paraffin)|
|PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH|
|AI595938; DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB; NEB177D; Nebulin; NEM2; nemaline myopathy type 2|
|KLH conjugated synthetic peptide derived from human Nebulin.|
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