Mouse Anti-NEB Recombinant Antibody (CBMAB-AO781LY)

Name: Mouse Anti-NEB Recombinant Antibody
SKU: CBMAB-AO781LY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Application

ELISA, WB

Immunogen

Chicken breast nebulin.

Specificity

Human, Monkey, Rat, Chicken

Antibody Isotype

IgG1

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!]

Format

Liquid

Buffer

2% BSA

Preservative

0.09% sodium azide

Concentration

1 mg/ml

Storage

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

More Infomation

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

Human	4703
Monkey	100430324
Rat	311029
Chicken	101748402

UniProt ID

Human	P20929
Monkey	A0A2K5U5W9
Rat	F1M1J2

Alternative Names

Nebulin; NEB

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.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

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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.).

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