Abstract:
: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.
journal_name
Trends Neuroscijournal_title
Trends in neurosciencesauthors
Shi L,Fu AK,Ip NYdoi
10.1016/j.tins.2012.04.005subject
Has Abstractpub_date
2012-07-01 00:00:00pages
441-53issue
7eissn
0166-2236issn
1878-108Xpii
S0166-2236(12)00056-2journal_volume
35pub_type
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