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Highest antigen sequence indentity to the following orthologs: Mouse (99%), Rat (99%).
This recombinant protein control fragment may be used for blocking experiments with the corresponding antibody, PA5-53457. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; Nebulin; nemaline myopathy type 2
基因别名: NEB; NEB177D; NEM2
UniProt ID: (Human) P20929
Entrez Gene ID: (Human) 4703