Muscle Skeletal Receptor Tyrosine Kinase (MUSK) Antibody
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Description
MUSK Antibody is a Rabbit Polyclonal antibody against MUSK. This gene encodes a muscle-specific tyrosine kinase receptor. The encoded protein may play a role in clustering of the acetylcholine receptor in the postsynaptic neuromuscular junction. Mutations in this gene have been associated with congenital myasthenic syndrome. Alternatively spliced transcript variants have been described.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Muscle Skeletal Receptor Tyrosine Kinase (MUSK) |
| Host | Rabbit |
| Reactivity | Mouse |
| Recommended Dilution | WB: 1/500 - 1/2000. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by affinity chromatography. |
| Size 1 | 60 µl |
| Size 2 | 120 µl |
| Size 3 | 200 µl |
| Form | Liquid |
| Tested Applications | WB |
| Buffer | PBS, pH 7.3, containing 0.05% Proclin-300, 50% glycerol. |
| 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 |
| NCBI Accession | NP_001159752.1 |
| Alias | CMS9,FADS,FADS1,Muscle-specific tyrosine-protein kinase receptor,Muscle skeletal receptor tyrosine-protein kinase |
| Background | Antibody anti-MUSK |
| Status | RUO |
| Note | Concentration: 1 mg/ml - |
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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