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首页> 外文期刊>The Journal of Neuroscience: The Official Journal of the Society for Neuroscience >Alterations in intrinsic membrane properties and the axon initial segment in a mouse model of Angelman syndrome.
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Alterations in intrinsic membrane properties and the axon initial segment in a mouse model of Angelman syndrome.

机译：固有膜性质和轴突初始节段在安格曼综合征小鼠模型中的变化。

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摘要

The axon initial segment (AIS) is the site of action potential initiation in neurons. Recent studies have demonstrated activity-dependent regulation of the AIS, including homeostatic changes in AIS length, membrane excitability, and the localization of voltage-gated Na(+) channels. The neurodevelopmental disorder Angelman syndrome (AS) is usually caused by the deletion of small portions of the maternal copy of chromosome 15, which includes the UBE3A gene. A mouse model of AS has been generated and these mice exhibit multiple neurological abnormalities similar to those observed in humans. We examined intrinsic properties of pyramidal neurons in hippocampal area CA1 from AS model mice and observed alterations in resting membrane potential, threshold potential, and action potential amplitude. The altered intrinsic properties in the AS mice were correlated with significant increases in the expression of the alpha1 subunit of Na/K-ATPase (alpha1-NaKA), the Na(+) channel NaV1.6, and the AIS anchoring protein ankyrin-G, as well as an increase in length of the AIS. These findings are the first evidence for pathology of intrinsic membrane properties and AIS-specific changes in AS, a neurodevelopmental disorder associated with autism.
机译：轴突起始节（AIS）是神经元中动作电位起始的部位。最近的研究表明AIS的活动依赖调节，包括AIS长度，膜兴奋性和电压门控Na（+）通道的位置的稳态变化。神经发育障碍Angelman综合征（AS）通常是由于删除了包含UBE3A基因的15号染色体母本的一小部分而引起的。已经产生了AS小鼠模型，并且这些小鼠表现出类似于在人类中观察到的多种神经异常。我们检查了AS模型小鼠海马区CA1中锥体神经元的内在特性，并观察了静息膜电位，阈值电位和动作电位振幅的变化。 AS小鼠内在特性的改变与Na / K-ATPase（alpha1-NaKA），Na（+）通道NaV1.6和AIS锚蛋白锚蛋白G的alpha1亚基表达的显着增加相关，以及AIS长度的增加。这些发现是内膜性质和AS的AIS特异性变化的病理学的第一个证据，AS是与自闭症相关的神经发育障碍。

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