How vital is sleep in Huntington’s disease?

Goodman, Anna O. G.; Barker, Roger A.

doi:10.1007/s00415-010-5517-4

Cited by 48 publications

(24 citation statements)

References 170 publications

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“…The results ( Table 3 ) clearly show that the mice exhibited a reduction of sleep early in their sleep cycle. This observation with the BACHD mice parallels clinical observations of a prolonged sleep latency in the HD patients (Aziz, et al, 2010, Cuturic, et al, 2009, Goodman and Barker, 2010). Future work using EEG recordings will be necessary to specifically examine if the BACHD mice show any change in sleep states or in the depth of sleep.…”

Section: Discussionsupporting

confidence: 84%

“…One likely consequence of these entrainment deficits is that the BACHD mice exhibit more activity during the time of the daily LD cycle when they should be sleeping. In HD patients, sleep disturbances are a common clinical complaint that impairs the quality of life (Goodman and Barker, 2010). To explore the sleep/wake patterns of the BACHD, we turned to behavioral measures of sleep (Campbell and Tobler, 1984, Loh, et al, 2010, Schwartz and Smale, 2005).…”

Section: Discussionmentioning

confidence: 99%

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Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease

Kudo

Schroeder

Loh

et al. 2011

Experimental Neurology

151

164

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Many patients with Huntington's disease (HD) exhibit disturbances in their daily cycle of sleep and wake as part of their symptoms. These patients have difficulty sleeping at night and staying awake during the day, which has a profound impact on the quality of life of the patients and their care-givers. In the present study, we examined diurnal and circadian rhythms of four models of HD including the BACHD, CAG 140 knock-in and R6/2 CAG 140 and R6/2 CAG 250 lines of mice. The BACHD and both R6/2 lines showed profound circadian phenotypes as measured by wheel-running activity. Focusing on the BACHD line for further analysis, the amplitude of the rhythms in the BACHD mice declined progressively with age. In addition, the circadian regulation of heart rate and body temperature in freely behaving BACHD mice were also disrupted. Furthermore, the distribution of sleep as well as the autonomic regulation of heart rate was disrupted in this HD model. To better understand the mechanistic underpinnings of the circadian disruption, we used electrophysiological tools to record from neurons within the central clock in the suprachiasmatic nucleus (SCN). The BACHD mice exhibit reduced rhythms in spontaneous electrical activity in SCN neurons. Interestingly, the expression of the clock gene PERIOD2 was not altered in the SCN of the BACHD line. Together, this data is consistent with the hypothesis that the HD mutations interfere with the expression of robust circadian rhythms in behavior and physiology. The data raise the possibility that the electrical activity within the central clock itself may be altered in this disease.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease

Kudo

Schroeder

Loh

et al. 2011

Experimental Neurology

151

164

View full text Add to dashboard Cite

show abstract

“…Patients with HD have progressive disordered sleep, with reduced sleep efficiency and total sleep duration [98,99]. Symptoms may include insomnia, RBD, advanced sleep phase, and reduced REM sleep [100].…”

Section: Sleep and Circadian Disruptions Are Common In Neurodegeneratmentioning

confidence: 99%

Circadian Rhythms, Sleep, and Disorders of Aging

Mattis

Sehgal

2016

Trends in Endocrinology & Metabolism

273

189

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Sleep:wake cycles are known to be disrupted in people with neurodegenerative disorders. These findings are now supported by data from animal models for some of these disorders, raising the question of whether the disrupted sleep/circadian regulation contributes to the loss of neural function. As circadian rhythms and sleep consolidation also break down with normal aging, changes in these may be part of what makes aging a risk factor for disorders like Alzheimer's disease. Mechanisms underlying the connection between circadian/sleep dysregulation and neurodegeneration remain unclear, but several recent studies provide interesting possibilities. While mechanistic analysis is underway, it is worth considering treatment of circadian/sleep disruption as a means to alleviate symptoms of neurodegenerative disorders.

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“…Patients with Huntington’s disease (HD) have sleep symptoms including advanced sleep phase, insomnia and reduced REM sleep (Arnulf et al, 2008; Goodman and Barker, 2010). Neuropathological analyses demonstrated that HD patients are depleted for many hypothalamic neuropeptides, i.e., AVP, oxytocin and hypocretin, hence disturbed regular sleep/awake daily cycle (Aziz et al, 2008; Gabery et al, 2010).…”

Section: Circadian Disruption and Neurodegenerative Diseasesmentioning

confidence: 99%

Physiological links of circadian clock and biological clock of aging

Liu

Chang

2017

Protein Cell

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Circadian rhythms orchestrate biochemical and physiological processes in living organisms to respond the day/night cycle. In mammals, nearly all cells hold self-sustained circadian clocks meanwhile couple the intrinsic rhythms to systemic changes in a hierarchical manner. The suprachiasmatic nucleus (SCN) of the hypothalamus functions as the master pacemaker to initiate daily synchronization according to the photoperiod, in turn determines the phase of peripheral cellular clocks through a variety of signaling relays, including endocrine rhythms and metabolic cycles. With aging, circadian desynchrony occurs at the expense of peripheral metabolic pathologies and central neurodegenerative disorders with sleep symptoms, and genetic ablation of circadian genes in model organisms resembled the aging-related features. Notably, a number of studies have linked longevity nutrient sensing pathways in modulating circadian clocks. Therapeutic strategies that bridge the nutrient sensing pathways and circadian clock might be rational designs to defy aging.

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How vital is sleep in Huntington’s disease?

Cited by 48 publications

References 170 publications

Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease

Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease

Circadian Rhythms, Sleep, and Disorders of Aging

Physiological links of circadian clock and biological clock of aging

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