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E-AB-12799

NEB Polyclonal Antibody

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£106.20

Was £118.00

SKU:
E-AB-12799
Additional Names:
DKFZp686C1456,FLJ11505,FLJ36536,FLJ39568,FLJ39584,NEB,NEB 177D,NEB177D,NEBU,NEM 2,NEM2,Nebulin
Application:
IHC
Concentration:
0.6 mg/ml
Purification:
Affinity Purified
Storage Conditions:
-20[o]C Avoid freeze/thaw cycles.
Supplier:
Elabscience
Host:
Rabbit
Reactivities:
Human
Buffer:
Phosphate buffered solution, pH 7.4, containing 0.05% stabilizer and 50% glycerol.
Immunogen:
Synthetic peptide of human NEB
Uniprot:
P20929
Synonyms:
AMC6;NEB177D;nebulin;NEM2;nemaline myopathy type 2
Extra Details:
This gene encodes nebulin, 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. The encoded protein contains approximately 30-amino acid long modules that can be classified into 7 types and other repeated modules. Protein isoform sizes vary from 600 to 800 kD due to alternative splicing that is tissue-, species-,and developmental stage-specific. Of the 183 exons in the nebulin gene, at least 43 are alternatively spliced, although exons 143 and 144 are not found in the same transcript. Of the several thousand transcript variants predicted for nebulin, the RefSeq Project has decided to create three representative RefSeq records. Mutations in this gene are associated with recessive nemaline myopathy.
Shipping Conditions:
Blue Ice