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Nebulin Rabbit anti-Human, Polyclonal, Bioss Non-distribution product as customer accommodation.

Rabbit Polyclonal Antibody

Manufacturer:  BiossSupplier Diversity Partner BS2401R

Catalog No. 50-198-4693


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Description

Description

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 kDa 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. Mutations in this gene are associated with recessive nemaline myopathy.
Specifications

Specifications

Nebulin
Immunofluorescence, Immunohistochemistry (Paraffin)
Unconjugated
NEB
Liquid
Rabbit
IgG
100 μL
-20°C
Primary
4703
NEB
1 μg/mL
PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
P20929
AI595938; DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB; NEB177D; Nebulin; NEM2; nemaline myopathy type 2
KLH conjugated synthetic peptide derived from human Nebulin.
Protein A
RUO
Antibody
Polyclonal
Human
SDS
Documents
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