PMID- 15034263
OWN - NLM
STAT- MEDLINE
DCOM- 20040702
LR  - 20061115
IS  - 0300-4864 (Print)
IS  - 0300-4864 (Linking)
VI  - 32
IP  - 5-8
DP  - 2003 Jun-Sep
TI  - The postsynaptic submembrane machinery at the neuromuscular junction: requirement 
      for rapsyn and the utrophin/dystrophin-associated complex.
PG  - 709-26
AB  - Neuromuscular synapse formation is brought about by a complex bi-directional 
      exchange of information between the innervating motor neuron and its target 
      skeletal muscle fiber. Agrin, a heparin sulfate proteoglycan, is released from 
      the motor nerve terminal to activate its muscle-specific kinase (MuSK) receptor 
      that leads to a second messenger cascade requiring rapsyn to ultimately bring 
      about AChR clustering in the muscle membrane. Rapsyn performs many functions in 
      skeletal muscle. First, rapsyn and AChRs co-target to the postsynatic apparatus. 
      Second, rapsyn may self associate to stabilize and promote AChR clustering. 
      Third, rapsyn is essential for AChR cluster formation. Fourth, rapsyn is required 
      to transduce the agrin-evoked MuSK phosphorylation signal to AChRs. Finally, 
      rapsyn links AChRs to the utrophin-associated complex, which appears to be 
      required for AChR stabilization as well as maturation of the neuromuscular 
      junction. Proteins within the utrophin-associated complex such as 
      alpha-dystrobrevin and alpha-syntrophin are also important for signaling events 
      that affect neuromuscular synapse stability and function. Here we review our 
      current understanding of the role of the postsynaptic-submembrane machinery 
      involving rapsyn and the utrophin-associated complex at the neuromuscular 
      synapse. In addition we briefly review how these studies of the neuromuscular 
      junction relate to GABAergic and glycinergic synapses in the CNS.
FAU - Banks, Glen B
AU  - Banks GB
AD  - Department of Physiology and Biophysics, University of Washington, Seattle, WA 
      98195-7290, USA.
FAU - Fuhrer, Christian
AU  - Fuhrer C
FAU - Adams, Marvin E
AU  - Adams ME
FAU - Froehner, Stanley C
AU  - Froehner SC
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PT  - Review
PL  - United States
TA  - J Neurocytol
JT  - Journal of neurocytology
JID - 0364620
RN  - 0 (Cytoskeletal Proteins)
RN  - 0 (Membrane Proteins)
RN  - 0 (Muscle Proteins)
RN  - 0 (Utrophin)
RN  - 0 (peripheral membrane protein 43K)
SB  - IM
MH  - Animals
MH  - Cytoskeletal Proteins/chemistry/genetics/*metabolism
MH  - Humans
MH  - Membrane Proteins/chemistry/genetics/*metabolism
MH  - Muscle Proteins/chemistry/genetics/*metabolism
MH  - Neuromuscular Junction/genetics/*metabolism
MH  - Synapses/genetics/metabolism
MH  - Synaptic Membranes/genetics/*metabolism
MH  - Utrophin
RF  - 195
EDAT- 2004/03/23 05:00
MHDA- 2004/07/03 05:00
CRDT- 2004/03/23 05:00
PHST- 2004/03/23 05:00 [pubmed]
PHST- 2004/07/03 05:00 [medline]
PHST- 2004/03/23 05:00 [entrez]
AID - 5256375 [pii]
AID - 10.1023/B:NEUR.0000020619.24681.2b [doi]
PST - ppublish
SO  - J Neurocytol. 2003 Jun-Sep;32(5-8):709-26. doi: 
      10.1023/B:NEUR.0000020619.24681.2b.