Hallengren Jada J, Vaden Ryan J
Department of Neurobiology and Evelyn F. McKnight Brain Institute, University of Alabama at Birmingham, Birmingham, Alabama
Department of Neurobiology and Evelyn F. McKnight Brain Institute, University of Alabama at Birmingham, Birmingham, Alabama.
J Neurophysiol. 2014 Jul 1;112(1):5-8. doi: 10.1152/jn.00760.2013. Epub 2014 Feb 5.
Angelman syndrome is a neurodevelopmental disorder characterized by intellectual disabilities, ataxia, and unusually happy affect. The hippocampal pyramidal cells of Angelman syndrome model mice have altered intrinsic membrane properties, which Kaphzan et al. (Cell Rep 4: 405-412, 2013) demonstrate can be corrected by genetic reduction of the α1-subunit of the sodium-potassium ATPase. Intriguingly, this manipulation also restores hippocampal long-term potentiation and learning. In this Neuro Forum, we discuss translational implications of this work and remaining questions left in its wake.
安格曼综合征是一种神经发育障碍,其特征为智力残疾、共济失调和异常愉悦的情绪。安格曼综合征模型小鼠的海马锥体细胞具有改变的内在膜特性,卡普赞等人(《细胞报告》4:405 - 412,2013年)证明,通过基因降低钠钾ATP酶的α1亚基可以纠正这种特性。有趣的是,这种操作还能恢复海马体的长时程增强和学习能力。在这个神经论坛中,我们讨论这项工作的转化意义以及随之而来的遗留问题。