Burden_2013_Cold.Spring.Harb.Perspect.Biol_5_a009167

Reference

Title : The role of MuSK in synapse formation and neuromuscular disease - Burden_2013_Cold.Spring.Harb.Perspect.Biol_5_a009167
Author(s) : Burden SJ , Yumoto N , Zhang W
Ref : Cold Spring Harb Perspect Biol , 5 :a009167 , 2013
Abstract :

Muscle-specific kinase (MuSK) is essential for each step in neuromuscular synapse formation. Before innervation, MuSK initiates postsynaptic differentiation, priming the muscle for synapse formation. Approaching motor axons recognize the primed, or prepatterned, region of muscle, causing motor axons to stop growing and differentiate into specialized nerve terminals. MuSK controls presynaptic differentiation by causing the clustering of Lrp4, which functions as a direct retrograde signal for presynaptic differentiation. 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. In addition, MuSK phosphorylation is stimulated by an inside-out ligand, docking protein-7 (Dok-7), which is recruited to tyrosine-phosphorylated MuSK and increases MuSK kinase activity. Mutations in MuSK and in genes that function in the MuSK signaling pathway, including Dok-7, cause congenital myasthenia, and autoantibodies to MuSK, Lrp4, and acetylcholine receptors are responsible for myasthenia gravis.

PubMedSearch : Burden_2013_Cold.Spring.Harb.Perspect.Biol_5_a009167
PubMedID: 23637281

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Citations formats

Burden SJ, Yumoto N, Zhang W (2013)
The role of MuSK in synapse formation and neuromuscular disease
Cold Spring Harb Perspect Biol 5 :a009167

Burden SJ, Yumoto N, Zhang W (2013)
Cold Spring Harb Perspect Biol 5 :a009167