Myasthenia gravis: subgroup classification and therapeutic strategies.
Bergen, Norway. In Lancet Neurol, Oct 2015
Myasthenia gravis is an autoimmune disease that is characterised by muscle weakness and fatigue, is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor, muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction.
The role of MuSK in synapse formation and neuromuscular disease.
New York City, United States. In Cold Spring Harb Perspect Biol, 2013
Developing synapses are stabilized by neuronal Agrin, which is released by motor nerve terminals and binds to Lrp4, a member of the low-density lipoprotein receptor family, stimulating further association between Lrp4 and MuSK and increasing MuSK kinase activity.
Lrp4 is a receptor for Agrin and forms a complex with MuSK.
New York City, United States. In Cell, 2008
MuSK, a receptor tyrosine kinase that is expressed in skeletal muscle, and Agrin, a motor neuron-derived ligand that stimulates MuSK phosphorylation, play critical roles in synaptic differentiation, as synapses do not form in their absence, and mutations in MuSK or downstream effectors are a major cause of a group of neuromuscular disorders, termed congenital myasthenic syndromes (CMS).