Muscle Skeletal Receptor Tyrosine Kinase (MUSK) Antibody
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Description
MuSK (for Muscle Specific Kinase) is a receptor tyrosine kinase required for the formation of the neuromuscular junction (NMJ). It induces cellular signaling by causing the addition of phosphate molecules to particular tyrosines on itself, and on proteins which bind the cytoplasmic domain of the receptor. It is activated by a nerve-derived proteoglycan called agrin. During development, the growing end of motor neuron axons secrete a protein called agrin.This protein binds to several receptors on the surface of skeletal muscle. The receptor which seems to be required for formation of the neuromuscular junction (NMJ), which comprises the nerve-muscle synapse is called MuSK. MUSK mutations lead to decreased agrin-dependent AchR aggregation, a critical step in the formation of the neuromuscular junction.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Muscle Skeletal Receptor Tyrosine Kinase (MUSK) |
| Host | Mouse |
| Reactivity | Human |
| Recommended Dilution | ELISA: 1/10000, IHC: 1/200 - 1/1000, IF/ICC: 1/200 - 1/1000. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Monoclonal |
| Conjugation | Unconjugated |
| Isotype | IgG1 |
| Purification | Unpurified ascites. |
| Size 1 | 100 µl |
| Form | Liquid |
| Tested Applications | ELISA, IHC, IF/ICC |
| Buffer | Ascitic fluid containing 0.03% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | O15146 |
| Gene ID | 4593 |
| OMIM | 208150 |
| Alias | CMS9,FADS,FADS1,Muscle-specific tyrosine-protein kinase receptor,Muscle skeletal receptor tyrosine-protein kinase |
| Background | Antibody anti-MUSK |
| Status | RUO |
| Note | Concentration: Not determined. - |
Descripción
Muscle-associated receptor tyrosine kinase (MUSK) is a critical protein in the formation and maintenance of the neuromuscular junction (NMJ), the synapse between motor neurons and muscle fibers. MUSK is essential for the proper functioning of the NMJ, playing a pivotal role in the signaling pathways that govern the assembly and stability of this specialized synapse. The NMJ is crucial for muscle contraction and overall motor function, and disruptions in MUSK function can lead to severe neuromuscular diseases. MUSK is encoded by the MUSK gene, located on chromosome 9 in humans. The protein belongs to the receptor tyrosine kinase family and is highly conserved across species, reflecting its essential role in muscle biology. MUSK activation is tightly regulated and involves interactions with several other proteins, including agrin, low-density lipoprotein receptor-related protein 4 (LRP4), and downstream effectors such as rapsyn.
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anti- MUSK antibody
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).
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