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anti-Mouse (Murine) MUSK Antikörper:
anti-Rat (Rattus) MUSK Antikörper:
anti-Human MUSK Antikörper:
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Human Polyclonal MUSK Primary Antibody für FACS, IHC (p) - ABIN392019
Chevessier, Faraut, Ravel-Chapuis, Richard, Gaudon, Bauché, Prioleau, Herbst, Goillot, Ioos, Azulay, Attarian, Leroy, Fournier, Legay, Schaeffer, Koenig, Fardeau, Eymard, Pouget, Hantaï: MUSK, a new target for mutations causing congenital myasthenic syndrome. in Human molecular genetics 2004
Show all 2 Pubmed References
Human Monoclonal MUSK Primary Antibody für ICC, IHC - ABIN969299
Boneva, Frenkian-Cuvelier, Bidault, Brenner, Berrih-Aknin: Major pathogenic effects of anti-MuSK antibodies in myasthenia gravis. in Journal of neuroimmunology 2006
prepatterning in zebrafish requires the MuSK Fz-like domain but not Lrp4 (zeige LRP4 Antikörper)
Data show that in vivo, wnt11r (zeige WNT11 Antikörper) and wnt4a initiate MuSK translocation from muscle membranes to recycling endosomes and that this transition is crucial for AChR accumulation at future synaptic sites.
Data suggest that a Wnt11r (zeige WNT11 Antikörper)-MuSK dependent, PCP (zeige PRCP Antikörper)-like pathway restricts neural crest cells to their segmental path.
AChR prepatterning and axonal guidance are spatio-temporally coordinated through common unplugged/MuSK signals, and that additional factor(s) restrict unplugged/MuSK signaling to a central muscle zone critical for establishing mid-muscle synaptogenesis
Zebrafish unplugged encodes a homolog of muscle-specific (zeige EIF3K Antikörper) kinase (MuSK); unlike mammalian MuSK, unplugged has only a limited role in neuromuscular synaptogenesis.
genetic evidence that neuromuscular synapse formation can occur in the absence of MuSK and that the combinatorial function of UnpFL/MuSK and dystroglycan generates diverse patterns of vertebrate neuromuscular innervation.
Gene expression profiling showed that MuSK was required for the BMP4 (zeige BMP4 Antikörper)-induced expression of a subset of genes in myoblasts, including regulator of G protein signaling 4 (Rgs4 (zeige RGS4 Antikörper)).
These mice showed only marginal activation of MuSK and died by 3 weeks of age apparently due to an abnormally small number and size of neuromuscular junction (NMJs).
data reveal that muscle skeletal receptor tyrosine kinase cysteine-rich domain is critical for neuromuscular junction formation and plays an unsuspected role in NMJ maintenance in adulthood.
Forced expression of Dok-7 (zeige DOK7 Antikörper) in muscle enhanced MuSK activation in mice lacking agrin (zeige AGRN Antikörper) or Lrp4 (zeige CORIN Antikörper) and restored midmuscle NMJ formation in agrin (zeige AGRN Antikörper)-deficient mice, but not in Lrp4 (zeige CORIN Antikörper)-deficient mice.
pathogenic IgG4 antibodies to MuSK bind to a structural epitope in the first Ig-like domain of MuSK, prevent binding between MuSK and Lrp4 (zeige CORIN Antikörper), and inhibit Agrin (zeige AGRN Antikörper)-stimulated MuSK phosphorylation.
MuSK colocalizes with actin and Arf6 (zeige ARF6 Antikörper) at the cell surface and during endosomal trafficking.
Increasing MuSK activity delays denervation and improves motor function in ALS mice.
Two classic synaptic signalling systems (neuregulin-1 (zeige NRG1 Antikörper) and neural agrin (zeige AGRN Antikörper)) converge upon MuSK to regulate postsynaptic differentiation.
Biglycan (zeige BGN Antikörper) binding to MuSK rescues the unstable acetylcholine receptor (zeige CHRNB1 Antikörper) clusters that are involved in neuromuscular junction formation and postsynaptic differentiation.
Data suggest that adult skeletal muscles harbour different endogenous levels of MuSK and that these levels determine the ability to form ectopic AChR clusters upon overexpression of agrin (zeige AGRN Antikörper) or MuSK.
Classical electromyography revealed the presence of myopathic changes more frequently in MuSK myasthenia gravis compared to acetylcholine receptor (zeige CHRNA1 Antikörper) myasthenia gravis
A Dutch founder mutation in MUSK causing fetal akinesia deformation sequence has been found in 14 fetuses.
To our knowledge, this is the first report showing that a mutation in MuSK is associated with Fetal akinesia deformation sequence syndrome
Immunosuppression attenuates the Th1 (zeige TH1L Antikörper) response in AChR-myasthenia gravis (MG) and MuSK-MG, but otherwise modulates immune responses in AChR-MG and MuSK-MG patients differentially.
HnRNP C, YB-1 (zeige YBX1 Antikörper) and hnRNP L (zeige HNRNPL Antikörper) coordinately enhance skipping of human MUSK exon 10 to generate a Wnt (zeige WNT2 Antikörper)-insensitive MuSK isoform.
MuSK myasthenia gravis IgG4 disrupts the interaction of LRP4 (zeige LRP4 Antikörper) with MuSK but both IgG4 and IgG1-3 can disperse preformed agrin (zeige AGRN Antikörper)-independent AChR clusters
[review] Recent discovery of two novel target proteins (MuSK and LRP4 (zeige LRP4 Antikörper)) has reduced the percentage of patients without known autoantibodies, although there are still some seronegative myasthenia gravis patients.
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.
HEp (zeige EPHB6 Antikörper)-2 M4 cells revealed a high specificity for the detection of MuSK autoantibodies from 25 patient sera.
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.
muscle, skeletal receptor tyrosine-protein kinase
, muscle, skeletal, receptor tyrosine kinase
, skeletal muscle receptor tyrosine kinase
, muscle, skeletal receptor tyrosine-protein kinase-like
, muscle localized kinase 2
, muscle, skeletal receptor tyrosine protein kinase
, muscle-specific kinase receptor
, muscle-specific protein kinase secretory isoform
, muscle-specific tyrosine protein kinase receptor
, muscle-specific tyrosine-protein kinase receptor
, muscle specific kinase (neural fold/somite kinase 1)
, muscle-specific tyrosine kinase receptor MuSK
, skeletal receptor tyrosine protein kinase