持续鞘内给予食欲素可抑制嗜睡症小鼠模型中的猝倒。

Continuous intrathecal orexin delivery inhibits cataplexy in a murine model of narcolepsy.

机构信息

International Institute for Integrative Sleep Medicine, University of Tsukuba, Tsukuba, 305-8575 Ibaraki, Japan.

Department of Neuropsychiatry, Akita University Graduate School of Medicine, 010-8543 Akita, Japan.

出版信息

Proc Natl Acad Sci U S A. 2018 Jun 5;115(23):6046-6051. doi: 10.1073/pnas.1722686115. Epub 2018 May 21.

Abstract

Narcolepsy-cataplexy is a chronic neurological disorder caused by loss of orexin (hypocretin)-producing neurons, associated with excessive daytime sleepiness, sleep attacks, cataplexy, sleep paralysis, hypnagogic hallucinations, and fragmentation of nighttime sleep. Currently, human narcolepsy is treated by providing symptomatic therapies, which can be associated with an array of side effects. Although peripherally administered orexin does not efficiently penetrate the blood-brain barrier, centrally delivered orexin can effectively alleviate narcoleptic symptoms in animal models. Chronic intrathecal drug infusion through an implantable pump is a clinically available strategy to treat a number of neurological diseases. Here we demonstrate that the narcoleptic symptoms of orexin knockout mice can be reversed by lumbar-level intrathecal orexin delivery. Orexin was delivered via a chronically implanted intrathecal catheter at the upper lumbar level. The computed tomographic scan confirmed that intrathecally administered contrast agent rapidly moved from the spinal cord to the brain. Intrathecally delivered orexin was detected in the brain by radioimmunoassay at levels comparable to endogenous orexin levels. Cataplexy and sleep-onset REM sleep were significantly decreased in orexin knockout mice during and long after slow infusion of orexin (1 nmol/1 µL/h). Sleep/wake states remained unchanged both quantitatively as well as qualitatively. Intrathecal orexin failed to induce any changes in double orexin receptor-1 and -2 knockout mice. This study supports the concept of intrathecal orexin delivery as a potential therapy for narcolepsy-cataplexy to improve the well-being of patients.

摘要

发作性睡病伴猝倒症是一种由食欲素(下丘脑分泌素)产生神经元缺失引起的慢性神经疾病,其特征是日间过度嗜睡、睡眠发作、猝倒、睡眠瘫痪、催眠幻觉和夜间睡眠碎片化。目前,发作性睡病患者采用的是对症治疗,可能会出现一系列副作用。尽管外周给予的食欲素不能有效地穿透血脑屏障,但中枢给予的食欲素可以有效地缓解动物模型中的发作性睡病症状。通过可植入的泵进行慢性鞘内药物输注是一种治疗多种神经疾病的临床可用策略。在这里,我们证明了通过腰椎水平鞘内给予食欲素可以逆转食欲素敲除小鼠的发作性睡病症状。通过慢性植入的鞘内导管在上腰椎水平给予食欲素。计算机断层扫描证实,鞘内给予的造影剂迅速从脊髓转移到大脑。通过放射免疫测定法在大脑中检测到与内源性食欲素水平相当的鞘内给予的食欲素。在缓慢输注食欲素(1 nmol/1 µL/h)期间和之后很长一段时间,食欲素敲除小鼠的猝倒和睡眠起始 REM 睡眠明显减少。睡眠/觉醒状态在数量和质量上都没有变化。鞘内给予食欲素对双食欲素受体-1 和 -2 敲除小鼠没有引起任何变化。这项研究支持鞘内给予食欲素作为治疗发作性睡病伴猝倒症的一种潜在疗法的概念,以改善患者的生活质量。

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