Sign in or Register   Sign in or Register
  |  

Mouse Anti-NEB Recombinant Antibody (1F4) (CBMAB-N1707-WJ)

This product is a Mouse antibody that recognizes NEB. The antibody 1F4 can be used for immunoassay techniques such as: WB, IP, ELISA.
See all NEB antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
1F4
Antibody Isotype
IgG1, κ
Application
WB, IP, ELISA

Basic Information

Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Nebulin
Introduction
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]
Entrez Gene ID
4703
UniProt ID
P20929
Alternative Names
Nebulin; Nemaline Myopathy Type 2; NEB177D; NEM2;
Function
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.
Biological Process
Cardiac muscle thin filament assembly Source: GO_Central
Muscle organ development Source: ProtInc
Regulation of actin filament length Source: UniProtKB
Somatic muscle development Source: UniProtKB
Cellular Location
Cytoskeleton
Other locations
Sarcomere
Involvement in disease
Nemaline myopathy 2 (NEM2):
A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-like or rod-shaped structures in muscle fibers on histologic examination.
Arthrogryposis multiplex congenita 6 (AMC6):
A form of arthrogryposis multiplex congenita, a developmental condition characterized by multiple joint contractures resulting from reduced or absent fetal movements. AMC6 is an autosomal recessive lethal form. Death usually occurs in utero or in infancy.

Huang, K., Li, Z., Zhong, D., Yang, Y., Yan, X., Feng, T., ... & Li, H. (2024). A Circular RNA Generated from Nebulin (NEB) Gene Splicing Promotes Skeletal Muscle Myogenesis in Cattle as Detected by a Multi‐Omics Approach. Advanced Science, 11(3), 2300702.

Moreno, C. A. M., Artilheiro, M. C., Fonseca, A. T. Q. S. M., Camelo, C. G., Medeiros, G. C. D., Sassi, F. C., ... & Zanoteli, E. (2023). Clinical manifestation of nebulin-associated nemaline myopathy. Neurology: Genetics, 9(1), e200056.

Gineste, C., Ogier, A. C., Varlet, I., Hourani, Z., Bernard, M., Granzier, H., ... & Gondin, J. (2020). In vivo characterization of skeletal muscle function in nebulin‐deficient mice. Muscle & nerve, 61(3), 416-424.

Yuen, M., & Ottenheijm, C. A. (2020). Nebulin: big protein with big responsibilities. Journal of Muscle Research and Cell Motility, 41, 103-124.

Kiss, B., Gohlke, J., Tonino, P., Hourani, Z., Kolb, J., Strom, J., ... & Granzier, H. (2020). Nebulin and Lmod2 are critical for specifying thin-filament length in skeletal muscle. Science advances, 6(46), eabc1992.

Laitila, J. M., McNamara, E. L., Wingate, C. D., Goullee, H., Ross, J. A., Taylor, R. L., ... & Nowak, K. J. (2020). Nebulin nemaline myopathy recapitulated in a compound heterozygous mouse model with both a missense and a nonsense mutation in Neb. Acta neuropathologica communications, 8, 1-19.

Laitila, J., Lehtonen, J., Lehtokari, V. L., Sagath, L., Wallgren‐Pettersson, C., Grönholm, M., & Pelin, K. (2019). A nebulin super‐repeat panel reveals stronger actin binding toward the ends of the super‐repeat region. Muscle & Nerve, 59(1), 116-121.

Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-NEB Recombinant Antibody (1F4)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare