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钠钾ATP酶成为天使综合征小鼠海马体表型中的一个作用因素。

Sodium-potassium ATPase emerges as a player in hippocampal phenotypes of Angelman syndrome mice.

作者信息

Hallengren Jada J, Vaden Ryan J

机构信息

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.

出版信息

J Neurophysiol. 2014 Jul 1;112(1):5-8. doi: 10.1152/jn.00760.2013. Epub 2014 Feb 5.

DOI:10.1152/jn.00760.2013
PMID:24501262
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4064391/
Abstract

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.

摘要

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。

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2
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Chronic α1-Na/K-ATPase inhibition reverses the elongation of the axon initial segment of the hippocampal CA1 pyramidal neurons in Angelman syndrome model mice.慢性α1-钠钾-ATP酶抑制可逆转天使综合征模型小鼠海马CA1锥体神经元轴突起始段的延长。
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本文引用的文献

1
Genetic reduction of the α1 subunit of Na/K-ATPase corrects multiple hippocampal phenotypes in Angelman syndrome.基因敲减 Na/K-ATPase 的α1 亚基可纠正 Angelman 综合征的多种海马表型。
Cell Rep. 2013 Aug 15;4(3):405-12. doi: 10.1016/j.celrep.2013.07.005. Epub 2013 Aug 1.
2
Evidence for a role of Nav1.6 in facilitating increases in neuronal hyperexcitability during epileptogenesis.证据表明,Nav1.6 在癫痫发生过程中促进神经元过度兴奋增加方面发挥作用。
J Neurophysiol. 2013 Sep;110(5):1144-57. doi: 10.1152/jn.00383.2013. Epub 2013 Jun 5.
3
Role of the ubiquitin ligase E6AP/UBE3A in controlling levels of the synaptic protein Arc.泛素连接酶 E6AP/UBE3A 在控制突触蛋白 Arc 水平中的作用。
Proc Natl Acad Sci U S A. 2013 May 28;110(22):8888-93. doi: 10.1073/pnas.1302792110. Epub 2013 May 13.
4
The Angelman syndrome protein Ube3a/E6AP is required for Golgi acidification and surface protein sialylation.Angelman 综合征相关蛋白 Ube3a/E6AP 对于 Golgi 酸化和表面蛋白唾液酸化是必需的。
J Neurosci. 2013 Feb 27;33(9):3799-814. doi: 10.1523/JNEUROSCI.1930-11.2013.
5
Impairment of TrkB-PSD-95 signaling in Angelman syndrome.Angelman 综合征中 TrkB-PSD-95 信号转导的损伤。
PLoS Biol. 2013;11(2):e1001478. doi: 10.1371/journal.pbio.1001478. Epub 2013 Feb 12.
6
Na+/K+-ATPase inhibition partially mimics the ethanol-induced increase of the Golgi cell-dependent component of the tonic GABAergic current in rat cerebellar granule cells.钠离子/钾离子 -ATP 酶抑制部分模拟了乙醇诱导的大鼠小脑颗粒细胞中高尔基细胞依赖性紧张性 GABA 电流成分的增加。
PLoS One. 2013;8(1):e55673. doi: 10.1371/journal.pone.0055673. Epub 2013 Jan 31.
7
Correcting deregulated Fxyd1 expression ameliorates a behavioral impairment in a mouse model of Rett syndrome.纠正失调的 Fxyd1 表达可改善 Rett 综合征小鼠模型的行为障碍。
Brain Res. 2013 Feb 16;1496:104-14. doi: 10.1016/j.brainres.2012.12.009. Epub 2012 Dec 14.
8
Decreased tonic inhibition in cerebellar granule cells causes motor dysfunction in a mouse model of Angelman syndrome.小脑颗粒细胞紧张性抑制减弱导致 Angelman 综合征小鼠模型运动功能障碍。
Sci Transl Med. 2012 Dec 5;4(163):163ra157. doi: 10.1126/scitranslmed.3004655.
9
Understanding the pathogenesis of Angelman syndrome through animal models.通过动物模型理解 Angelman 综合征的发病机制。
Neural Plast. 2012;2012:710943. doi: 10.1155/2012/710943. Epub 2012 Jul 8.
10
Role of estrogen and progesterone in the modulation of CNG-A1 and Na/K+-ATPase expression in the renal cortex.
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