Nebulin (NEB) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC
Recommend Dilution	IHC: 50-200 Positive control: Human lung cancer Predicted cell location: Cytoplasm
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	Synthetic peptide corresponding to a region derived from 118-132 amino acids of human nebulin
Formulation	PBS pH7.3, 0.05% NaN3, 50% glycerol
Purification	Antigen affinity purification
Conjugation	Unconjugated
Storage Condition	Store at -20°C as received.
Gene Name	nebulin
Database Link	NP_004534 Entrez Gene 4703 Human UniProt ID P20929
Background	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.
Synonyms	NEB177D; NEM2
Reference Data
Protein Families	Druggable Genome

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