catalog number :
MBS850308
products full name :
MUSK Antibody
products short name :
MUSK
products name syn :
muscle; skeletal; receptor tyrosine kinase
other names :
muscle, skeletal receptor tyrosine-protein kinase isoform 2; Muscle, skeletal receptor tyrosine-protein kinase; muscle, skeletal receptor tyrosine-protein kinase; muscle-specific kinase receptor; muscle-specific tyrosine-protein kinase receptor; muscle, skeletal, receptor tyrosine kinase; Muscle-specific tyrosine-protein kinase receptor; MuSK; Muscle-specific kinase receptor
products gene name :
MUSK
other gene names :
MUSK; MUSK; MuSK; Muscle-specific kinase receptor
uniprot entry name :
MUSK_HUMAN
form :
Ascitic fluid containing 0.03% sodium azide.
concentration :
100ug/100ul
storage stability :
Store at 4 degree C. For long term storage, store at -20 degree C
tested application :
Immunohistochemistry (IHC), Immunofluorescence (IF), ELISA (EIA)
app notes :
IHC: 1/200 - 1/1000, IF: 1/200 - 1/1000, ELISA: Propose dilution 1/10000. Not yet tested in other applications. Determining optimal working dilutions by titration test.
other info2 :
Immunogen: Purified recombinant extracellular fragment of human MUSK (aa24-209) fused with hIgGFc tag expressed in HEK293 cell line. Species: Mouse IgG1
products categories :
Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells; Autophagy antibody
products description :
Background: 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.
ncbi acc num :
NP_001159752.1
ncbi gb acc num :
NM_001166280.1
ncbi pathways :
ECM Proteoglycans Pathway (833812); Extracellular Matrix Organization Pathway (576262)
ncbi summary :
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.[provided by RefSeq, Oct 2009]
uniprot summary :
MUSK: a receptor tyrosine kinase that is essential for the establishment and maintenance of the neuromuscular junction (NMJ). Its activation by agrin, a neuronally derived heparan-sulfate proteoglycan, and the agrin receptor (LRP4), leads to clustering of acetylcholine receptors on the postsynaptic side of the NMJ. Its activation by agrin requires Dok7, which interacts with the cytoplasmic portion of MuSK and activates its tyrosine kinase activity. Protein type: EC 2.7.10.1; Protein kinase, TK; Membrane protein, integral; Protein kinase, tyrosine (receptor); Kinase, protein; TK group; Musk family. Chromosomal Location of Human Ortholog: 9q31.3-q32. Cellular Component: postsynaptic membrane; integral to plasma membrane; neuromuscular junction; cell junction; receptor complex. Molecular Function: protein binding; protein-tyrosine kinase activity; transmembrane receptor protein tyrosine kinase activity; ATP binding. Biological Process: extracellular matrix organization and biogenesis; peptidyl-tyrosine phosphorylation; regulation of transcription, DNA-dependent; positive regulation of neuron apoptosis; protein amino acid autophosphorylation; multicellular organismal development; regulation of synaptic growth at neuromuscular junction; positive regulation of protein amino acid phosphorylation; cell differentiation; neuromuscular junction development; transmembrane receptor protein tyrosine kinase signaling pathway; memory. Disease: Myasthenic Syndrome, Congenital, 9, Associated With Acetylcholine Receptor Deficiency; Fetal Akinesia Deformation Sequence