NEB Antibody (PACO18739)
- SKU:
- PACO18739
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- IHC
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | NEB Antibody (PACO18739) |
| Antibody SKU: | PACO18739 |
| Size: | 50ul |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, IHC |
| Recommended Dilutions: | ELISA:1:2000-1:5000, IHC:1:50-1:200 |
| Species Reactivity: | Human |
| Immunogen: | Synthetic peptide of human NEB |
| Form: | Liquid |
| Storage Buffer: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
| Purification Method: | Antigen affinity purification |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | The image on the left is immunohistochemistry of paraffin-embedded Human lung cancer tissue using PACO18739(NEB Antibody) at dilution 1/80, on the right is treated with synthetic peptide. (Original magnification: x200). |
| 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: | nebulin |
| UniProt Protein Function: | NEB: This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Binds and stabilize F-actin. Defects in NEB are the cause of nemaline myopathy type 2 (NEM2). A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination. |
| UniProt Protein Details: | Protein type:Cytoskeletal Chromosomal Location of Human Ortholog: 2q22 Cellular Component: cytosol; Z disc; actin cytoskeleton Molecular Function:protein binding; structural constituent of muscle; actin binding Biological Process: muscle development; regulation of actin filament length; somatic muscle development; muscle filament sliding Disease: Nemaline Myopathy 2 |
| NCBI Summary: | 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. [provided by RefSeq, Sep 2009] |
| UniProt Code: | P20929 |
| NCBI GenInfo Identifier: | 635377472 |
| NCBI Gene ID: | 4703 |
| NCBI Accession: | P20929.5 |
| UniProt Related Accession: | P20929 |
| Molecular Weight: | Observed: 773 kDaPredicted: 773 kDa |
| NCBI Full Name: | Nebulin |
| NCBI Synonym Full Names: | nebulin |
| NCBI Official Symbol: | NEB |
| NCBI Official Synonym Symbols: | NEM2; NEB177D |
| NCBI Protein Information: | nebulin |
| UniProt Protein Name: | Nebulin |
| Protein Family: | Nebulin |
| UniProt Gene Name: | NEB |
| UniProt Entry Name: | NEBU_HUMAN |
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