Western Blot (WB)
(Anti- Nebulin antibody, MBS175052, Western blottingAll lanes: Anti Nebulin (MBS175052) at 0.5ug/mlWB: Mouse Skeletal Muscle Tissue Lysate at 50ugPredicted bind size: 773KDObserved bind size: 773KD )
Mouse anti-Chicken Nebulin Monoclonal Antibody | anti-NEB antibody
Anti-Nebulin antibody (monoclonal)
Western Blot (WB)
(Anti- Nebulin antibody, MBS175052, Western blottingAll lanes: Anti Nebulin (MBS175052) at 0.5ug/mlWB: Mouse Skeletal Muscle Tissue Lysate at 50ugPredicted bind size: 773KDObserved bind size: 773KD )
Western Blot (WB)
(Anti- Nebulin antibody, MBS175052, Western blottingAll lanes: Anti Nebulin (MBS175052) at 0.5ug/mlWB: Mouse Skeletal Muscle Tissue Lysate at 50ugPredicted bind size: 773KDObserved bind size: 773KD )
Background: Nebulin is 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 and its size varies from 600 to 800 kD in a manner that is tissue-, species-, and developmental stage-specific. The nebulin gene contains 183 exons in a 249-kb genomic region. Nebulin is mapped to chromosome 2. Nebulin is a giant filamentous protein specific for vertebrate skeletal muscles. Mutations in the nebulin gene associated with autosomal recessive nemaline myopathy.
NCBI and Uniprot Product Information
NCBI 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 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 Description
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.
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
Research Articles on NEB
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Product Notes
The NEB neb (Catalog #AAA175052) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Anti-Nebulin antibody (monoclonal) reacts with Chicken and may cross-react with other species as described in the data sheet. AAA Biotech's Nebulin can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the NEB neb for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Nebulin, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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