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. 2021 Feb;99(2):529-544.
doi: 10.1002/jnr.24730. Epub 2020 Sep 28.

Sleep problems in Rett syndrome animal models: A systematic review

Xinyan Zhang  1 , Jian-Sheng Lin  1 , Karen Spruyt  1
Affiliations

Sleep problems in Rett syndrome animal models: A systematic review

Xinyan Zhang et al. J Neurosci Res. 2021 Feb.

Abstract

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.

Keywords: Drosophila; Rett syndrome; circadian rhythm; monkey; mouse; sleep.

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References

REFERENCES

    1. Aldrich, M. S., Garofalo, E. A., & Drury, I. (1990). Epileptiform abnormalities during sleep in Rett syndrome. Electroencephalography and Clinical Neurophysiology, 75(5), 365-370. https://doi.org/10.1016/0013-4694(90)90081-T
    1. Amaddeo, A., De Sanctis, L., Arroyo, J. O., Khirani, S., Bahi-Buisson, N., & Fauroux, B. (2019). Polysomnographic findings in Rett syndrome. European Journal of Paediatric Neurology, 23(1), 214-221. https://doi.org/10.1016/j.ejpn.2018.09.003
    1. Amendola, E., Zhan, Y., Mattucci, C., Castroflorio, E., Calcagno, E., Fuchs, C., … Gross, C. T. (2014). Mapping pathological phenotypes in a mouse model of CDKL5 disorder. PLoS ONE, 9(5), e91613. https://doi.org/10.1371/journal.pone.0091613
    1. Amir, R. E., Van Den Veyver, I. B., Wan, M., Tran, C. Q., Francke, U., & Zoghbi, H. Y. (1999). Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl- CpG-binding protein 2. Nature Genetics, 23(2), 185-188. https://doi.org/10.1038/13810
    1. Ammanuel, S., Chan, W. C., Adler, D. A., Lakshamanan, B. M., Gupta, S. S., Ewen, J. B., … Kadam, S. D. (2015). Heightened delta power during slow-wave-sleep in patients with rett syndrome associated with poor sleep efficiency. PLoS ONE, 10(10), e0138113. https://doi.org/10.1371/journal.pone.0138113

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