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Publication : Ephexin1 is required for structural maturation and neurotransmission at the neuromuscular junction.

First Author  Shi L Year  2010
Journal  Neuron Volume  65
Issue  2 Pages  204-16
PubMed ID  20152127 Mgi Jnum  J:167656
Mgi Id  MGI:4868684 Doi  10.1016/j.neuron.2010.01.012
Citation  Shi L, et al. (2010) Ephexin1 is required for structural maturation and neurotransmission at the neuromuscular junction. Neuron 65(2):204-16
abstractText  The maturation of neuromuscular junctions (NMJs) requires the topological transformation of postsynaptic acetylcholine receptor (AChR)-containing structures from a simple plaque to an elaborate structure composed of pretzel-like branches. This maturation process results in the precise apposition of the presynaptic and postsynaptic specializations. However, little is known about the molecular mechanisms underlying the plaque-to-pretzel transition of AChR clusters. In this study, we identify an essential role for the RhoGEF ephexin1 in the maturation of AChR clusters. Adult ephexin1(-/-) mice exhibit severe muscle weakness and impaired synaptic transmission at the NMJ. Intriguingly, when ephexin1 expression is deficient in vivo, the NMJ fails to mature into the pretzel-like shape, and such abnormalities can be rescued by re-expression of ephexin1. We further demonstrate that ephexin1 regulates the stability of AChR clusters in a RhoA-dependent manner. Taken together, our findings reveal an indispensible role for ephexin1 in regulating the structural maturation and neurotransmission of NMJs.
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