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Mouse Anti-MUSK Recombinant Antibody (4E5) (CBMAB-A5691-LY)

The product is antibody recognizes MUSK. The antibody 4E5 immunoassay techniques such as: WB, ELISA.
See all MUSK antibodies
Published Data
Fig.2 Increased expression of MUC5AC in hyperplastic polyp, similar to MUC2. Immunostaining with M1. Bars 5 100 mm.
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Summary

Host Animal
Mouse
Specificity
Human
Clone
4E5
Antibody Isotype
IgG2a, κ
Application
WB, ELISA

Basic Information

Immunogen
MUSK (NP_005583, 301 a.a. ~ 400 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human
Antibody Isotype
IgG2a, 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!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Muscle Associated Receptor Tyrosine Kinase
Introduction
Intercellular communication is often mediated by receptors on the surface of one cell that recognize and are activated by specific protein ligands released by other cells. Members of one class of cell surface receptors, receptor tyrosine kinases (RTKs), are characterized by having a cytoplasmic domain containing intrinsic tyrosine kinase activity. This kinase activity is regulated by the binding of a cognate ligand to the extracellular portion of the receptor. DeChiara et al. (1996) [PubMed 8653786] noted that the RTKs, known to be expressed in cell type-specific fashions, play a role critical for the growth and differentiation of those cell types. For example, members of the neural-specific TRK family that recognize nerve growth factor are absolutely required for the survival and development of discrete neuronal subpopulations, and the receptor tyrosine kinases TIE1 (MIM 600222) and TIE2 (MIM 600221) play a critical role in the development of normal blood vessels.[supplied by OMIM
Entrez Gene ID
4593
UniProt ID
O15146
Alternative Names
MGC126323; MGC126324
Function
Receptor tyrosine kinase which plays a central role in the formation and the maintenance of the neuromuscular junction (NMJ), the synapse between the motor neuron and the skeletal muscle (PubMed:25537362).

Recruitment of AGRIN by LRP4 to the MUSK signaling complex induces phosphorylation and activation of MUSK, the kinase of the complex. The activation of MUSK in myotubes regulates the formation of NMJs through the regulation of different processes including the specific expression of genes in subsynaptic nuclei, the reorganization of the actin cytoskeleton and the clustering of the acetylcholine receptors (AChR) in the postsynaptic membrane. May regulate AChR phosphorylation and clustering through activation of ABL1 and Src family kinases which in turn regulate MUSK. DVL1 and PAK1 that form a ternary complex with MUSK are also important for MUSK-dependent regulation of AChR clustering. May positively regulate Rho family GTPases through FNTA. Mediates the phosphorylation of FNTA which promotes prenylation, recruitment to membranes and activation of RAC1 a regulator of the actin cytoskeleton and of gene expression. Other effectors of the MUSK signaling include DNAJA3 which functions downstream of MUSK. May also play a role within the central nervous system by mediating cholinergic responses, synaptic plasticity and memory formation (By similarity).
Biological Process
Cell differentiation Source: UniProtKB-KW
Memory Source: UniProtKB
Neuromuscular junction development Source: UniProtKB
Positive regulation of gene expression Source: UniProtKB
Positive regulation of kinase activity Source: GO_Central
Positive regulation of protein geranylgeranylation Source: UniProtKB
Positive regulation of protein phosphorylation Source: UniProtKB
Protein autophosphorylation Source: UniProtKB
Regulation of synaptic assembly at neuromuscular junction Source: UniProtKB
Skeletal muscle acetylcholine-gated channel clustering Source: UniProtKB
Transmembrane receptor protein tyrosine kinase signaling pathway Source: GO_Central
Cellular Location
Plasma membrane
postsynaptic cell membrane
Note: Colocalizes with acetylcholine receptors (AChR) to the postsynaptic cell membrane of the neuromuscular junction.
Involvement in disease
Myasthenic syndrome, congenital, 9, associated with acetylcholine receptor deficiency (CMS9):
A form of congenital myasthenic syndrome, a group of disorders characterized by failure of neuromuscular transmission, including pre-synaptic, synaptic, and post-synaptic disorders that are not of autoimmune origin. Clinical features are easy fatigability and muscle weakness affecting the axial and limb muscles (with hypotonia in early-onset forms), the ocular muscles (leading to ptosis and ophthalmoplegia), and the facial and bulbar musculature (affecting sucking and swallowing, and leading to dysphonia). The symptoms fluctuate and worsen with physical effort. CMS9 is a disorder of postsynaptic neuromuscular transmission, due to deficiency of AChR at the endplate that results in low amplitude of the miniature endplate potential and current.
Fetal akinesia deformation sequence 1 (FADS1):
A clinically and genetically heterogeneous group of disorders with congenital malformations related to impaired fetal movement. Clinical features include fetal akinesia, intrauterine growth retardation, polyhydramnios, arthrogryposis, pulmonary hypoplasia, craniofacial abnormalities, and cryptorchidism. FADS1 inheritance is autosomal recessive.
Topology
Extracellular: 24-495
Helical: 496-516
Cytoplasmic: 517-869
PTM
Ubiquitinated by PDZRN3. Ubiquitination promotes endocytosis and lysosomal degradation (By similarity).
Phosphorylated (By similarity). Phosphorylation is induced by AGRIN in a LRP4-dependent manner (By similarity). Autophosphorylated (PubMed:25029443). Autophosphorylation at Tyr-554 is required for interaction with DOK7 which in turn stimulates the phosphorylation and the activation of MUSK (By similarity).
Neddylated.

Xie, T., Xu, G., Liu, Y., Quade, B., Lin, W., & Bai, X. C. (2023). Structural insights into the assembly of the agrin/LRP4/MuSK signaling complex. Proceedings of the National Academy of Sciences, 120(23), e2300453120.

Prömer, J., Barresi, C., & Herbst, R. (2023). From phosphorylation to phenotype–Recent key findings on kinase regulation, downstream signaling and disease surrounding the receptor tyrosine kinase MuSK. Cellular Signalling, 110584.

Mori, S., Suzuki, S., Konishi, T., Kawaguchi, N., Kishi, M., Kuwabara, S., ... & Shigemoto, K. (2023). Proteolytic ectodomain shedding of muscle-specific tyrosine kinase in myasthenia gravis. Experimental Neurology, 361, 114300.

Budayeva, H. G., Sengupta-Ghosh, A., Phu, L., Moffat, J. G., Ayalon, G., & Kirkpatrick, D. S. (2022). Phosphoproteome profiling of the receptor tyrosine kinase MuSK identifies tyrosine phosphorylation of Rab GTPases. Molecular & Cellular Proteomics, 21(4).

Chen, Y., Guan, M., Yu, F., Yang, Z., Yi, W., Huang, X., ... & Lai, F. (2022). Protein Tyrosine Phosphatase Receptor Type R (PTPRR) Reduces AChR Clustering by Dephosphorylating MuSK. Disease Markers, 2022.

Herbst, R. (2020). MuSk function during health and disease. Neuroscience Letters, 716, 134676.

Rodríguez Cruz, P. M., Cossins, J., Cheung, J., Maxwell, S., Jayawant, S., Herbst, R., ... & Beeson, D. (2020). Congenital myasthenic syndrome due to mutations in MUSK suggests that the level of MuSK phosphorylation is crucial for governing synaptic structure. Human mutation, 41(3), 619-631.

Summala, K. (2019). Biochemical and functional characterization of the interaction between two receptor tyrosine kinases, ROR1 and MuSK.

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

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