Anti-Nebulin Antibody (Monoclonal, NB2), Carrier-free

About this product

Boster Bio Anti-Nebulin Antibody (Monoclonal, NB2) catalog # MA1069. Tested in WB applications. This antibody reacts with Chicken.

Specifications

Catalog number

MA1069-carrier-free

Size / count

100 µg

Dry ice

No

Hazardous

No

Discontinued

Yes

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.

Synonyms

Nebulin;NEB;

Gene Name

Nebulin

Gene ID

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

UniProt

P20929

Host

Mouse

Reactivity

Chicken

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

Chicken breast nebulin.

Clonality

Monoclonal

Clone

Clone: NB2

Tissue Specificity

Muscle specific. Located in the thin filament of striated muscle.

Applications

WB

Purification

Ascites

Concentration

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

Form

Lyophilized

Reconstitution

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

Function

This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Binds and stabilize F-actin.

Components

Mouse ascites fluid, 1.2% sodium acetate, 2mg BSA, with 0.01mg NaN3 as preservative.

References & Citations

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.

Storage Conditions

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.

Calculated Molecular Weight

772914 MW

Observed Molecular Weight

773 kDa

Fragment

Mouse IgG1

Specificity

No cross reactivity with other proteins.

Applications Notes

Western blot, 1-2 μg/ml, Chicken<br>

Subcellular Location

Cytoplasm, myofibril, sarcomere. Cytoplasm, cytoskeleton.

Sequence Similarities

Contains 178 nebulin repeats.

Protein Name

Nebulin