PMID- 17081697
OWN - NLM
STAT- MEDLINE
DCOM- 20070306
LR  - 20161124
IS  - 0306-4522 (Print)
IS  - 0306-4522 (Linking)
VI  - 143
IP  - 3
DP  - 2006 Dec
TI  - Delayed synapsing muscles are more severely affected in an experimental model of 
      MuSK-induced myasthenia gravis.
PG  - 655-9
AB  - Myasthenia gravis can be induced in mice by injecting the extracellular domain of 
      rat muscle-specific kinase (MuSK), a transmembrane receptor tyrosine kinase 
      involved in agrin signaling at the neuromuscular junction. About 5-10% of human 
      myasthenia gravis patients have autoantibodies against MuSK. Here we have 
      examined mouse neuromuscular junctions following MuSK immunization in two groups 
      of muscles that can be distinguished on the basis of the timing of neuromuscular 
      synaptogenesis and their response to perturbation of agrin signaling. We used 
      confocal microscopy to characterize the distribution and expression of nicotinic 
      acetylcoline receptors and of two presynaptic makers, neurofilament protein and 
      synaptophysin. We observed disruption of neuromuscular junctions in all muscles 
      examined in this model of myasthenia gravis. However delayed-synapsing muscles, 
      including the diaphragm, sternomastoid and tibialis posterior, were significantly 
      more severely affected than fast-synapsing muscles, including the intercostal, 
      adductor longus and tibialis anterior. These results suggest a basis for the 
      differential susceptibility of muscles in different classes of myasthenia gravis 
      patients, including patients with autoantibodies against MuSK.
FAU - Xu, K
AU  - Xu K
AD  - Department of Biology and Biochemistry, University of Houston, Houston, TX 
      77204-5001, USA.
FAU - Jha, S
AU  - Jha S
FAU - Hoch, W
AU  - Hoch W
FAU - Dryer, S E
AU  - Dryer SE
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20061101
PL  - United States
TA  - Neuroscience
JT  - Neuroscience
JID - 7605074
RN  - 0 (Agrin)
RN  - 0 (Neurofilament Proteins)
RN  - 0 (Receptors, Cholinergic)
RN  - 0 (Receptors, Nicotinic)
RN  - 0 (Synaptophysin)
RN  - EC 2.7.10.1 (MuSK protein, rat)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Agrin/metabolism
MH  - Animals
MH  - Disease Models, Animal
MH  - Female
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Microscopy, Confocal/methods
MH  - Muscles/metabolism/*pathology
MH  - Myasthenia Gravis/chemically induced/*pathology
MH  - Neurofilament Proteins/metabolism
MH  - Neuromuscular Junction/*metabolism
MH  - Rats
MH  - *Receptor Protein-Tyrosine Kinases
MH  - *Receptors, Cholinergic
MH  - Receptors, Nicotinic/metabolism
MH  - Synaptophysin/metabolism
MH  - Time Factors
EDAT- 2006/11/04 09:00
MHDA- 2007/03/07 09:00
CRDT- 2006/11/04 09:00
PHST- 2006/07/10 00:00 [received]
PHST- 2006/09/12 00:00 [revised]
PHST- 2006/09/16 00:00 [accepted]
PHST- 2006/11/04 09:00 [pubmed]
PHST- 2007/03/07 09:00 [medline]
PHST- 2006/11/04 09:00 [entrez]
AID - S0306-4522(06)01250-4 [pii]
AID - 10.1016/j.neuroscience.2006.09.022 [doi]
PST - ppublish
SO  - Neuroscience. 2006 Dec;143(3):655-9. doi: 10.1016/j.neuroscience.2006.09.022. 
      Epub 2006 Nov 1.