期刊
JOURNAL OF NEUROSCIENCE
卷 30, 期 3, 页码 849-857出版社
SOC NEUROSCIENCE
DOI: 10.1523/JNEUROSCI.4496-09.2010
关键词
-
资金
- GENAME
- Spanish Ministry of Education and Science [BFU2007-61171, BES2005-6739]
Low levels of survival motor neuron (SMN) protein result in spinal muscular atrophy (SMA), a severe genetic disease characterized by motor impairment and premature lethality. Although SMN is a ubiquitous protein, motor neurons are much more vulnerable to low levels of SMN than other cells. To gain insight into the pathogenesis of SMA, we have compared synaptic function of motor terminals in wild-type and severe SMA mice at different ages and in two proximal muscles. Our results show that mutant muscle fibers fire normal action potentials and that multi-innervated terminals are functional. By studying the characteristics of the three main components of synaptic transmission in nerve terminals (spontaneous, evoked, and asynchronous release), we found that the kinetics of the postsynaptic potentials are slowed and evoked neurotransmitter release is decreased by similar to 55%. In addition, asynchronous release is increased similar to 300%, indicating an anomalous augmentation of intraterminal bulk Ca2+ during repetitive stimulation. Together, these results show that the reduction of SMN affects synaptic maturation, evoked release, and regulation of intraterminal Ca2+ levels.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据