Journal
TRENDS IN NEUROSCIENCES
Volume 35, Issue 7, Pages 441-453Publisher
ELSEVIER SCIENCE LONDON
DOI: 10.1016/j.tins.2012.04.005
Keywords
synapse; acetylcholine receptor; neuromuscular disorder; congenital myasthenic syndrome; agrin; MuSK
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Funding
- Research Grants Council of Hong Kong [6421/05 M, 661007, 661109, 660810, HKUST6/CRF/08]
- University Grants Committee [AoE/B-15/01-II]
- Hong Kong Jockey Club
- National Natural Science Foundation of China [81101015]
- Bureau of Science and Information Technology of Guangzhou Municipality [2011J2200048]
- Croucher Foundation
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The vertebrate neuromuscular junction (NMJ), a peripheral synapse formed between motoneuron and skeletal muscle, is characterized by a protracted postnatal period of maturation and life-long maintenance. In neuromuscular disorders such as congenital myasthenic syndromes (CMSs), disruptions of NMJ maturation and/or maintenance are frequently observed. In particular, defective neuromuscular transmission associated with structural and molecular abnormalities at the pre- and postsynaptic membranes, as well as at the synaptic cleft, has been reported in these patients. Here, we review recent advances in the understanding of molecular and cellular events that mediate NMJ maturation and maintenance. The underlying regulatory mechanisms, including key molecular regulators at the presynaptic nerve terminal, synaptic cleft, and postsynaptic muscle membrane, are discussed.
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