MuSK Antibody (1429CT456.173.44): sc-517346

MuSK Antibody (1429CT456.173.44) is a mouse monoclonal IgG1 antibody that detects MuSK in mouse and rat samples through applications such as western blotting (WB), immunofluorescence (IF), and immunohistochemistry with paraffin-embedded sections (IHCP). MuSK , or muscle-specific kinase, plays a crucial role in the development and maintenance of the neuromuscular junction, where MuSK is essential for the clustering of acetylcholine receptors and the formation of synaptic connections between motor neurons and muscle fibers. MuSK function is vital for effective neuromuscular transmission, and MuSK dysregulation can lead to various neuromuscular disorders, including congenital myasthenic syndromes. MuSK is predominantly expressed in skeletal muscle, particularly at the neuromuscular junction, where MuSK facilitates communication between nerve and muscle cells, ensuring proper muscle contraction and coordination. The human MuSK gene is located on chromosome 9q31.3, overlapping a region associated with Fukuyama muscular dystrophy, highlighting MuSK significance in muscle health and disease. Anti-MuSK antibody (1429CT456.173.44) is an invaluable tool for researchers studying muscle biology and related pathologies.

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MuSK Antibody (1429CT456.173.44) References:

1. The agrin/muscle-specific kinase pathway: new targets for autoimmune and genetic disorders at the neuromuscular junction.  |  Liyanage, Y., et al. 2002. Muscle Nerve. 25: 4-16. PMID: 11754179
2. Muscle-specific kinase myasthenia gravis IgG4 autoantibodies cause severe neuromuscular junction dysfunction in mice.  |  Klooster, R., et al. 2012. Brain. 135: 1081-101. PMID: 22396395
3. Muscle specific kinase autoantibodies cause synaptic failure through progressive wastage of postsynaptic acetylcholine receptors.  |  Morsch, M., et al. 2012. Exp Neurol. 237: 286-95. PMID: 22789393
4. Mechanisms associated with the pathogenicity of antibodies against muscle-specific kinase in myasthenia gravis.  |  Mori, S. and Shigemoto, K. 2013. Autoimmun Rev. 12: 912-7. PMID: 23537506
5. Muscle-specific kinase (MuSK) autoantibodies suppress the MuSK pathway and ACh receptor retention at the mouse neuromuscular junction.  |  Ghazanfari, N., et al. 2014. J Physiol. 592: 2881-97. PMID: 24860174
6. Muscle specific kinase (MuSK) activation preserves neuromuscular junctions in the diaphragm but is not sufficient to provide a functional benefit in the SOD1G93A mouse model of ALS.  |  Sengupta-Ghosh, A., et al. 2019. Neurobiol Dis. 124: 340-352. PMID: 30528255
7. Efgartigimod improves muscle weakness in a mouse model for muscle-specific kinase myasthenia gravis.  |  Huijbers, MG., et al. 2019. Exp Neurol. 317: 133-143. PMID: 30851266
8. Muscle specific kinase protects dystrophic mdx mouse muscles from eccentric contraction-induced loss of force-producing capacity.  |  Trajanovska, S., et al. 2019. J Physiol. 597: 4831-4850. PMID: 31340406
9. Vector-mediated expression of muscle specific kinase restores specific force to muscles in the mdx mouse model of Duchenne muscular dystrophy.  |  Ban, J., et al. 2021. Exp Physiol. 106: 1794-1805. PMID: 34114278
10. Dimerization of the muscle-specific kinase induces tyrosine phosphorylation of acetylcholine receptors and their aggregation on the surface of myotubes.  |  Hopf, C. and Hoch, W. 1998. J Biol Chem. 273: 6467-73. PMID: 9497380

The preferred MuSK antibody is MuSK Antibody (1-YD2): sc-134398.