Sleep Alterations in a Mouse Model of Spinocerebellar Ataxia Type 3.
Sleep Alterations in a Mouse Model of Spinocerebellar Ataxia Type 3.
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脊髓小脑性共济失调3型（SCA3）是一种神经退行性疾病，表现为几个脑区进行性神经元丧失和广泛的运动和非运动症状，包括共济失调和睡眠改变。虽然已知睡眠障碍在其他神经退行性疾病中发挥病理生理作用，但其对SCA3的影响尚不清楚。使用光谱测量，我们试图定量表征SCA3转基因小鼠的睡眠脑电图（EEG），并确认疾病表型。我们首先测量了18 - 31周龄纯合子SCA3 YACMJD84.2小鼠和非转基因野生型同窝小鼠在开灯和关灯期间的运动表型。在小鼠26-36周龄时，连续3天植入电极，获得12小时（授时数0-12）的脑电图记录。基于脑电图的光谱分析显示，与野生型幼崽相比，SCA3纯合子小鼠表现出：(1)快速眼动睡眠（REM）持续时间增加，所有睡眠和清醒状态都出现碎片化；（ii）快速眼动和非快速眼动（NREM）期间更高的β功率振荡；以及（iii）在REM和尾流期间额外的频谱功率带变化。我们的数据显示，纯合子SCA3小鼠的睡眠结构和脑电图频谱功率失调，表明常见的睡眠相关病因可能是小鼠和人类SCA3表型的基础。
Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder showing progressive neuronal loss in several brain areas and a broad spectrum of motor and non-motor symptoms, including ataxia and altered sleep. While sleep disturbances are known to play pathophysiologic roles in other neurodegenerative disorders, their impact on SCA3 is unknown. Using spectrographic measurements, we sought to quantitatively characterize sleep electroencephalography (EEG) in SCA3 transgenic mice with confirmed disease phenotype. We first measured motor phenotypes in 18–31-week-old homozygous SCA3 YACMJD84.2 mice and non-transgenic wild-type littermate mice during lights-on and lights-off periods. We next implanted electrodes to obtain 12-h (zeitgeber time 0-12) EEG recordings for three consecutive days when the mice were 26–36 weeks old. EEG-based spectroscopy showed that compared to wild-type littermates, SCA3 homozygous mice display: (i) increased duration of rapid-eye movement sleep (REM) and fragmentation in all sleep and wake states; (ii) higher beta power oscillations during REM and non-REM (NREM); and (iii) additional spectral power band alterations during REM and wake. Our data show that sleep architecture and EEG spectral power are dysregulated in homozygous SCA3 mice, indicating that common sleep-related etiologic factors may underlie mouse and human SCA3 phenotypes.
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