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Recombinant Human Muscle, skeletal receptor tyrosine-protein kinase(MUSK),partial

ArtNr CSB-EP015241HU-200
Hersteller Cusabio
Menge 200 ug
Quantity options 1 mg 10 ug 100 ug 20 ug 200 ug 50 ug 500 ug
Kategorie
Typ Proteins Recombinant
Format Liquid or Lyophilized powder
Specific against other
Host E.coli
Konjugat/Tag GST
Purity Greater than 90% as determined by SDS-PAGE.
Sequence LPKAPVITTPLETVDALVEEVATFMCAVESYPQPE ISWTRNKILIKLFDTRYSIRENGQLLTILSVEDSD DGIYCCTANNGVGGAVESCGALQVKMKPKITRPPI NVKIIEGLKAVLPCTTMGNPKPSVSWIKGDSPLRE NSRIAVLESGSLRIHNVQKEDAGQYRCVAKNSLGT AYSKVVKLEVEEESEPEQDTKVFARILRAPESHNV TFGSFVTLHCTATGIPVPTITWIENGNAVSSGSIQ ESV
Protein Familie Protein kinase superfamily, Tyr protein kinase family
Citations Salbutamol-responsive limb-girdle congenital myasthenic syndrome due to a novel missense mutation and heteroallelic deletion in MUSK.Gallenmuller C., Muller-Felber W., Dusl M., Stucka R., Guergueltcheva V., Blaschek A., von der Hagen M., Huebner A., Muller J.S., Lochmuller H., Abicht A.Neuromuscul. Disord. 24:31-35(2014)
ECLASS 10.1 32160409
ECLASS 11.0 32160409
UNSPSC 12352202
Alias Muscle-specific tyrosine-protein kinase receptor ;MuSK ;Muscle-specific kinase receptor
Lieferbar
Manufacturer - Conjugate / Tag
N-terminal GST-tagged
Storage Conditions
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20C/-80C. The shelf life of lyophilized form is 12 months at -20C/-80C.
Molecular Weight
78.8 kDa
General Research Areas
Signal Transduction
Relevance
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 . 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 mbrane. 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 mbranes 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 syst by mediating cholinergic responses, synaptic plasticity and mory formation .1 Publication
Expression Region
24-495aa
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4C for up to one week.
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
Subcellular Location
Cell junction, synapse, postsynaptic cell membrane, Single-pass type I membrane protein
Involvement in disease
Myasthenic syndrome, congenital, 9, associated with acetylcholine receptor deficiency (CMS9); Fetal akinesia deformation sequence (FADS)
Gene Names
MUSK
Sequence Info
partial of Isoform 2
Organism
Homo sapiens (Human)
Storage Buffer
Tris-based buffer, 50% glycerol

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Alle Produkte sind nur für Forschungszwecke bestimmt. Nicht für den menschlichen, tierärztlichen oder therapeutischen Gebrauch.

Menge: 200 ug
Lieferbar: In stock
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