Human Muscle Skeletal Receptor Tyrosine Kinase (MUSK) ELISA Kit

Instructions
Code CSB-E15804h
Size 96T,5×96T,10×96T
See More Details 24T ELISA kits trial application
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Product Details

Target Name muscle, skeletal, receptor tyrosine kinase
Alternative Names CMS9 ELISA Kit; FADS ELISA Kit; MDK 4 ELISA Kit; MDK4 ELISA Kit; MGC126323 ELISA Kit; MGC126324 ELISA Kit; Muscle ELISA Kit; Muscle associated receptor tyrosine kinase ELISA Kit; Muscle skeletal receptor tyrosine kinase ELISA Kit; Muscle skeletal receptor tyrosine protein kinase ELISA Kit; Muscle specific kinase receptor ELISA Kit; Muscle specific tyrosine kinase receptor ELISA Kit; Muscle specific tyrosine protein kinase receptor ELISA Kit; Muscle-specific kinase receptor ELISA Kit; Muscle-specific tyrosine-protein kinase receptor ELISA Kit; MuSK ELISA Kit; MUSK_HUMAN ELISA Kit; Neural fold somite kinase 1 ELISA Kit; Neural fold somite kinase 2 ELISA Kit; Neural fold somite kinase 3 ELISA Kit; Neural fold somite kinase1 ELISA Kit; Neural fold somite kinase2 ELISA Kit; Neural fold somite kinase3 ELISA Kit; Nsk 1 ELISA Kit; Nsk 2 ELISA Kit; Nsk 3 ELISA Kit; Nsk1 ELISA Kit; Nsk2 ELISA Kit; Nsk3 ELISA Kit; Receptor tyrosine kinase MuSK ELISA Kit; Skeletal muscle receptor tyrosine kinase ELISA Kit; skeletal receptor tyrosine-protein kinase ELISA Kit
Abbreviation MUSK
Uniprot No. O15146
Species Homo sapiens (Human)
Sample Types serum, plasma, tissue homogenates
Detection Range 1.56 pg/mL-100 pg/mL
Sensitivity 0.39 pg/mL
Assay Time 1-5h
Sample Volume 50-100ul
Detection Wavelength 450 nm
Research Area Signal Transduction
Assay Principle quantitative
Measurement Sandwich
Precision
Intra-assay Precision (Precision within an assay): CV%<8%
Three samples of known concentration were tested twenty times on one plate to assess.
Inter-assay Precision (Precision between assays): CV%<10%
Three samples of known concentration were tested in twenty assays to assess.
Linearity
To assess the linearity of the assay, samples were spiked with high concentrations of human MUSK in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
SampleSerum(n=4)
1:100Average %97
Range %91-103
1:200Average %89
Range %84-95
1:400Average %95
Range %90-99
1:800Average %103
Range %99-109
Recovery
The recovery of human MUSK spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
Sample TypeAverage % RecoveryRange
Serum (n=5) 9085-99
EDTA plasma (n=4)9894-102
Typical Data
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
pg/mlOD1OD2AverageCorrected
1002.675 2.552 2.614 2.510
502.226 2.376 2.301 2.197
251.672 1.594 1.633 1.529
12.50.792 0.816 0.804 0.700
6.250.446 0.427 0.437 0.333
3.120.236 0.231 0.234 0.130
1.560.180 0.189 0.185 0.081
00.106 0.102 0.104
Troubleshooting
and FAQs
ELISA kit FAQs
Storage Store at 2-8°C. Please refer to protocol.
Lead Time 3-5 working days

Target Data

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
Gene References into Functions
  1. Gene expression profiling showed that MuSK was required for the BMP4-induced expression of a subset of genes in myoblasts, including regulator of G protein signaling 4 (Rgs4). PMID: 27601729
  2. Classical electromyography revealed the presence of myopathic changes more frequently in MuSK myasthenia gravis compared to acetylcholine receptor myasthenia gravis PMID: 26778359
  3. A Dutch founder mutation in MUSK causing fetal akinesia deformation sequence has been found in 14 fetuses. PMID: 25537362
  4. To our knowledge, this is the first report showing that a mutation in MuSK is associated with Fetal akinesia deformation sequence syndrome PMID: 25612909
  5. Immunosuppression attenuates the Th1 response in AChR-myasthenia gravis (MG) and MuSK-MG, but otherwise modulates immune responses in AChR-MG and MuSK-MG patients differentially. PMID: 25893403
  6. HnRNP C, YB-1 and hnRNP L coordinately enhance skipping of human MUSK exon 10 to generate a Wnt-insensitive MuSK isoform. PMID: 25354590
  7. MuSK myasthenia gravis IgG4 disrupts the interaction of LRP4 with MuSK but both IgG4 and IgG1-3 can disperse preformed agrin-independent AChR clusters PMID: 24244707
  8. [review] Recent discovery of two novel target proteins (MuSK and LRP4) has reduced the percentage of patients without known autoantibodies, although there are still some seronegative myasthenia gravis patients. PMID: 24530233
  9. Identification of a novel missense mutation c.114T > A; p.Asp38Glu heteroallelic to a genomic deletion encompassing exons 2-3 of MUSK that explain a limb-girdle congenital myasthenic syndrome in two affected brothers of a Turkish family. PMID: 24183479
  10. HEp-2 M4 cells revealed a high specificity for the detection of MuSK autoantibodies from 25 patient sera. PMID: 24416182
  11. This study provides a replication of the highly significant associations of both HLA-DRB1( *)16,-DRB1( *)14 and -DQB1( *)05 with MuSK-MG. PMID: 23993985
  12. pathogenic IgG4 antibodies to MuSK bind to a structural epitope in the first Ig-like domain of MuSK, prevent binding between MuSK and Lrp4, and inhibit Agrin-stimulated MuSK phosphorylation. PMID: 24297891
  13. We proved that the missense mutations in ColQ-CTD cause endplate AChE deficiency by compromising ColQ-MuSK interaction at the NMJ. PMID: 23553736
  14. MUSK is associated with a small but variable subgroup of distinct phenotypes in Thai patients with myasthenia gravis who have MUSK autoantibodies. PMID: 23352351
  15. MUSK antibodies may induce phenotypically disruptive actions at the neuromuscular junction by binding acetylcholinesterase (AChE) via its collagen tail, producing a reduction in synaptic AChE activity. PMID: 23720161
  16. MuSK is activated in a complex spatio-temporal manner to cluster acetylcholine receptors on the postsynaptic (muscle) side of the synapse and to induce differentiation of the nerve terminal on the presynaptic side. (Review) PMID: 23467009
  17. We report a novel mutation in MUSK leading to a Congenital myasthenic syndromes PMID: 23326516
  18. MuSK kinase activity is necessary for substrate-dependent acetylcholine receptor cluster formation PMID: 22210232
  19. Two family cases are reported that transmit MuSK antibody myasthenia gravis to the offspring by different maternal mechanisms. PMID: 21386774
  20. The ability of immobilized MuSK extracellular domain to remove practically all anti-MuSK antibodies from patients' sera should prove invaluable for development of an antigen-specific therapeutic approach for MuSK myasthenia gravis. PMID: 21993075
  21. Lrp4 is a cis-acting ligand for MuSK PMID: 21969364
  22. The importance of MuSK as a synapse organiser is highlighted by cases of autoimmune myasthenia gravis in which MuSK autoantibodies can deplete MuSK from the postsynaptic membrane, leading to complete disassembly of the adult neuromuscular junction. PMID: 20974278
  23. these findings demonstrate that missense mutations in MUSK can result in a severe form of congenital myasthenic syndrome and indicate that the inability of MuSK mutants to interact with Dok-7. PMID: 20371544
  24. Anti-MuSK protein positive patients have more predominantly bulbar involvement and had more severe myasthenia gravis. PMID: 19327804
  25. analysis of regulation of MuSK expression by a novel signaling pathway PMID: 12885777
  26. Missense mutation does not affect MuSK catalytic kinase activity but diminishes expression and stability. PMID: 15496425
  27. Thus, an agrin/MuSK complex may form part of a motor neuron stop signal involved in "reverse signaling" to the motor neuron. PMID: 15691710
  28. A low-molecular weight isoform of muscle-specific receptor tyrosine kinase in human sperm localized in the flagellar mid-piece region. PMID: 16487930
  29. Dok-7 is essential for neuromuscular synaptogenesis through its interaction with MuSK PMID: 16794080
  30. Altogether, these results indicate that anti-MuSK Abs could be pathogenic by contributing to the muscle atrophy in MuSK+ MG patients. PMID: 16857268
  31. muscle-specific receptor tyrosine kinase activation and binding to dystroglycan are regulated by alternative mRNA splicing of agrin PMID: 17012237
  32. Testing of human myotubes for the presence and activation of MuSK by exposing them to laminin. PMID: 17192614
  33. We describe a case of epileptic seizures secondary to myasthenia gravis caused by autoantibodies to the MUSK receptor. These autoantibodies affected the brain as well. PMID: 17661994
  34. the COOH-terminal NES and Src homology 2 target motifs play key roles in Dok-7/MuSK signaling for neuromuscular synaptogenesis. PMID: 18165682
  35. We describe a transient neonatal myasthenic syndrome with anti-musk antibodies. PMID: 18378885
  36. IgG from anti-MuSK-positive patients can cause myasthenia gravis when injected into mice. PMID: 18384168
  37. single-fiber electromyography of distal limb muscles tends to have a lower yield of abnormality in MuSK-antibody-positive patients than either acetylcholine receptor-antibody-positive or seronegative myasthenia gravis PMID: 18567855
  38. Thymectomy is mostly considered scarcely effective; however, at present, no firm conclusions can be drawn on its role in the treatment of this form of myasthenia gravis PMID: 18567856
  39. Anti-MuSK antibodies influence the activity of MuSK molecules without reducing their number, thereby diminishing the size of the endplate and affecting the functioning of acetylcholine receptors. PMID: 19745065
  40. This study report a family known so far with a congenital myasthenic syndromes due to a mutation in the MUSK gene. PMID: 19949040

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Involvement in disease Myasthenic syndrome, congenital, 9, associated with acetylcholine receptor deficiency (CMS9); Fetal akinesia deformation sequence (FADS)
Subcellular Location Cell junction, synapse, postsynaptic cell membrane, Single-pass type I membrane protein
Protein Families Protein kinase superfamily, Tyr protein kinase family
Database Links

HGNC: 7525

OMIM: 208150

KEGG: hsa:4593

STRING: 9606.ENSP00000363571

UniGene: Hs.521653

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