Monoclonal Anti-Nebulin

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SPECIFICATIONS

Price

200.00 USD

Catalog Number

MA1069

Size

100ug/vial

Applications

WB

Reactivities

Ch/Bird

Form

Lyophilized

Format

1.2% sodium acetate or neutral PBS. If 1ml of PBS is used, the antibody concentration will be 100ug/ml.

Gene Id

Human(4703), Mouse(17996 ), Rat(311029 ), Chicken(374027)

Swiss Prot

Human(P20929)

Storage Temp

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Target Synonym

NEM2; NEB177D

Primary Protein

Nebulin

Scientific 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.