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Target antigen
Nebulin
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Clonality
Monoclonal antibody
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Clone
NB2
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Raised in
mice
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Type of the antibody
IgG1 monoclonal antibody
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Product form
freeze-dried
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Reacts with species
Human, mouse, rat
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Analyses
WB
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Immunogen
Chicken breast nebulin.
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Product configuration
Mouse ascites fluid, 1.2% Na acetate, 2mg BSA, with 0.01mg NaN3 as preservative.
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Purification
Ascites
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Solubilization
The powdered antibody should be dissolved in 1ml of PBS buffer to achieve final concentration of 100ug/ml.
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Storage condtions
Keep the Nebulin Antibody ( NB2)at minus twenty degrees Celsius for 1 year. The ready-to-use solutions can be stored at four degrees Celsius for a month. Our specialsits recommend to freeze the aliquotes at minus twenty degrees Celsius for long-term application. Multiple procedures of freezing and thawing influence the specifity and reactivity of the antibody in a negative way.
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Tips
The Nebulin Antibody ( NB2) did not cross-reacted with other proteins during the test procedures. This antobdy is intended to be used for research analyses and it is not applicale for in vitro diagnostics.
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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.
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Related articles
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.
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Gene Name
NEB
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Protein Name
Nebulin
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Gene Full Name
nebulin
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Synonyms
NEM2|NEB177D
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Uniprot ID
Human(P20929)
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Entrez GeneID
Human(4703), Mouse(17996), Rat(311029), Chicken(374027)
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Properties
If you buy Antibodies supplied by boster they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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French translation
anticorps
