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雷特综合征(一种女性神经遗传性疾病)动物模型中的呼吸异常

Breathing abnormalities in animal models of Rett syndrome a female neurogenetic disorder.

作者信息

Jiang Chun, Cui Ningren, Zhong Weiwei, Johnson Christopher M, Wu Yang

机构信息

Department of Biology, Georgia State University, Atlanta, USA.

Department of Biology, Georgia State University, Atlanta, USA.

出版信息

Respir Physiol Neurobiol. 2017 Nov;245:45-52. doi: 10.1016/j.resp.2016.11.011. Epub 2016 Nov 21.

Abstract

A characteristic feature of Rett syndrome (RTT) is abnormal breathing accompanied by several other neurological and cognitive disorders. Since RTT rodent models became available, studies have begun shedding insight into the breathing abnormalities at behavioral, cellular and molecular levels. Defects are found in several groups of brainstem neurons involved in respiratory control, and potential neural mechanisms have been suggested. The findings in animal models are helpful in therapeutic strategies for people with RTT with respect to lowering sudden and unexpected death, preventing secondary developmental consequences, and improving the quality of lives.

摘要

雷特综合征(RTT)的一个特征是呼吸异常,并伴有其他几种神经和认知障碍。自从有了RTT啮齿动物模型后,研究开始在行为、细胞和分子水平上深入了解呼吸异常情况。在参与呼吸控制的几组脑干神经元中发现了缺陷,并提出了潜在的神经机制。动物模型中的研究结果有助于为RTT患者制定治疗策略,以降低突然和意外死亡的风险、预防继发性发育后果并改善生活质量。

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