Anti-Nebulin Antibody (Monoclonal, NB2)

SKU: MA1069

Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Chicken

Applications: WB

Application Details: Western blot, 1-2 μg/ml, Chicken

Gene Name: NEB

Specificity: No cross reactivity with other proteins.

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.

Immunogen: Chicken breast nebulin.

Clonality: Monoclonal

Contents: Mouse IgG in stabilizing components, 1.2% sodium acetate and 0.01mg NaN3. *This antibody is supplied in a stabilized formulation.
Compatibility with conjugation reactions depends on the chemistry of the conjugation method used.
For conjugation methods that are not compatible with the stabilizing components present in this formulation, a carrier-free antibody format is required.

Purification: Ascites

Reconstitution: Add 1ml of PBS buffer will yield a concentration of 100ug/ml.

Reference: 1. Limongi, M. Z.; Pelliccia, F.; Rocchi, A. : Assignment of the human nebulin gene (NEB) to chromosome band 2q24.2 and the alpha-1 (III) collagen gene (COL3A1) to chromosome band 2q32.2 by in situ hybridization: the FRA2G common fragile site lies between the two genes in the 2q31 band. Cytogenet. Cell Genet. 77: 259-260, 1997. 2. Labeit, S.; Kolmerer, B. : The complete primary structure of human nebulin and its correlation to muscle structure. J. Molec. Biol. 248: 308-315, 1995. 3. Pelin, K.; Hilpela, P.; Donner, K.; Sewry, C.; Akkari, P. A.; Wilton, S. D.; Wattanasirichaigoon, D.; Bang, M.-L.; Centner, T.; Hanefeld, F.; Odent, S.; Fardeau, M.; Urtizberea, J. A.; Muntoni, F.; Dubowitz, V.; Beggs, A. H.; Laing, N. G.; Labeit, S.; de la Chapelle, A.; Wallgren-Pettersson, C. : Mutations in the nebulin gene associated with autosomal recessive nemaline myopathy. Proc. Nat. Acad. Sci. 96: 2305-2310, 1999.

Uniprot ID: P20929

Host: Mouse

Concentration: Adding 1 ml of PBS buffer will yield a concentration of 100 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins

Isotype: Mouse IgG1

Phospho_site:

Clone Number: Clone: NB2

Observed Molecular Weight: 773 kDa

Calculated Molecular Weight: 772914 MW

Gene ID: Human(4703), Mouse(17996), Rat(311029), Chicken(374027)

Protein Name: Nebulin

Gene Full Name: nebulin

Synonyms: NEB; NEB177D; nebulin; NEM2