- Catalog number
- MA1069
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- 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
- NEB
- 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
- Field of Research
- Actin Binding Proteins, Actin, etc., Cytoskeleton, Cytoskeleton/ECM, Microfilaments, Signal Transduction
- 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.
- 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°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 772914 MW
- Observed Molecular Weight
- 773 kDa
- Specificity
- No cross reactivity with other proteins.
- Gene Name Synonym
- Nebulin
- Subcellular Location
- Cytoplasm, myofibril, sarcomere. Cytoplasm, cytoskeleton.
- Sequence Similarities
- Contains 178 nebulin repeats.
- Protein Name
- Nebulin
- Isotype
- Mouse IgG1
- Contents
- Mouse ascites fluid, 1.2% sodium acetate, 2mg BSA, with 0.01mg NaN3 as preservative.
