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Sleep problems in Rett syndrome animal models: A systematic review.
Journal of Neuroscience Research ( IF 2.9 ) Pub Date : 2020-09-28 , DOI: 10.1002/jnr.24730
Xinyan Zhang 1 , Jian-Sheng Lin 1 , Karen Spruyt 1
Affiliation  

Due to the discovery of Rett Syndrome (RTT) genetic mutations, animal models have been developed. Sleep research in RTT animal models may unravel novel neural mechanisms for this severe neurodevelopmental heritable rare disease. In this systematic literature review we summarize the findings on sleep research of 13 studies in animal models of RTT. We found disturbed efficacy and continuity of sleep in all genetically mutated models of mice, cynomolgus monkeys, and Drosophila. Models presented highly fragmented sleep with distinct differences in 24‐hr sleep/wake cyclicity and circadian arrhythmicity. Overall, animal models mimic sleep complaints reported in individuals with RTT. However, contrary to human studies, in mutant mice, attenuated sleep delta waves, and sleep apneas in non‐rapid eye movement sleep were reported. Future studies may focus on sleep structure and EEG alterations, potential central mechanisms involved in sleep fragmentation and the occurrence of sleep apnea across different sleep stages. Given that locomotor dysfunction is characteristic of individuals with RTT, studies may consider to integrate its potential impact on the behavioral analysis of sleep.

中文翻译:

雷特综合征动物模型的睡眠问题:系统评价。

由于雷特综合征(RTT)基因突变的发现,已经开发了动物模型。RTT 动物模型的睡眠研究可能会揭示这种严重的神经发育遗传性罕见疾病的新神经机制。在这篇系统的文献综述中,我们总结了 13 项 RTT 动物模型研究的睡眠研究结果。我们发现在小鼠、食蟹猴和果蝇的所有基因突变模型中,睡眠的功效和连续性受到干扰. 模型呈现高度碎片化的睡眠,24 小时睡眠/觉醒周期和昼夜节律性有明显差异。总体而言,动物模型模拟了 RTT 患者报告的睡眠问题。然而,与人类研究相反,据报道,在突变小鼠中,非快速眼动睡眠中的睡眠δ波减弱和睡眠呼吸暂停。未来的研究可能会集中在睡眠结构和脑电图的改变、睡眠片段化的潜在中心机制以及不同睡眠阶段睡眠呼吸暂停的发生。鉴于运动功能障碍是 RTT 患者的特征,研究可能会考虑整合其对睡眠行为分析的潜在影响。
更新日期:2020-09-28
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