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000276320 041__ $$aEnglish
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000276320 1001_ $$00000-0002-1623-7091$$aGuillot, Simon J$$b0
000276320 245__ $$aEarly-onset sleep alterations found in patients with amyotrophic lateral sclerosis are ameliorated by orexin antagonist in mouse models.
000276320 260__ $$aWashington, DC$$bAAAS$$c2025
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000276320 520__ $$aSleep alterations have been described in several neurodegenerative diseases yet are currently poorly characterized in amyotrophic lateral sclerosis (ALS). This study investigates sleep macroarchitecture and related hypothalamic signaling disruptions in ALS. Using polysomnography, we found that both patients with ALS as well as asymptomatic C9ORF72 and SOD1 mutation carriers exhibited increased wakefulness and reduced non-rapid eye movement sleep. Increased wakefulness correlated with diminished cognitive performance in both clinical cohorts. Similar changes in sleep macroarchitecture were observed in three ALS mouse models (Sod1G86R, FusΔNLS/+, and TDP43Q331K). A single oral administration of a dual-orexin receptor antagonist or intracerebroventricular delivery of melanin-concentrating hormone (MCH) through an osmotic pump over 15 days partially normalized sleep patterns in mouse models. MCH treatment did not extend the survival of Sod1G86R mice but did decrease the loss of lumbar motor neurons. These findings suggest MCH and orexin signaling as potential targets to treat sleep alterations that arise in early stages of the disease.
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000276320 650_7 $$2NLM Chemicals$$aOrexins
000276320 650_7 $$2NLM Chemicals$$aMelanins
000276320 650_7 $$067382-96-1$$2NLM Chemicals$$amelanin-concentrating hormone
000276320 650_7 $$2NLM Chemicals$$aHypothalamic Hormones
000276320 650_7 $$2NLM Chemicals$$aPituitary Hormones
000276320 650_7 $$2NLM Chemicals$$aOrexin Receptors
000276320 650_7 $$0EC 1.15.1.1$$2NLM Chemicals$$aSuperoxide Dismutase-1
000276320 650_2 $$2MeSH$$aAnimals
000276320 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: drug therapy
000276320 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: pathology
000276320 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: metabolism
000276320 650_2 $$2MeSH$$aOrexins: metabolism
000276320 650_2 $$2MeSH$$aDisease Models, Animal
000276320 650_2 $$2MeSH$$aHumans
000276320 650_2 $$2MeSH$$aSleep: drug effects
000276320 650_2 $$2MeSH$$aMale
000276320 650_2 $$2MeSH$$aMelanins: metabolism
000276320 650_2 $$2MeSH$$aMice
000276320 650_2 $$2MeSH$$aWakefulness: drug effects
000276320 650_2 $$2MeSH$$aFemale
000276320 650_2 $$2MeSH$$aMotor Neurons: drug effects
000276320 650_2 $$2MeSH$$aMotor Neurons: pathology
000276320 650_2 $$2MeSH$$aMotor Neurons: metabolism
000276320 650_2 $$2MeSH$$aHypothalamic Hormones: metabolism
000276320 650_2 $$2MeSH$$aPituitary Hormones: metabolism
000276320 650_2 $$2MeSH$$aOrexin Receptors: metabolism
000276320 650_2 $$2MeSH$$aSuperoxide Dismutase-1: metabolism
000276320 650_2 $$2MeSH$$aSuperoxide Dismutase-1: genetics
000276320 650_2 $$2MeSH$$aMice, Transgenic
000276320 7001_ $$0P:(DE-2719)9002237$$aLang, Christina$$b1
000276320 7001_ $$00009-0001-6623-0648$$aSimonot, Marie$$b2
000276320 7001_ $$00009-0004-8901-662X$$aBeckett, Daniel$$b3
000276320 7001_ $$0P:(DE-2719)9002172$$aLulé, Dorothée$$b4
000276320 7001_ $$00009-0002-8088-3386$$aBalz, Luisa T$$b5
000276320 7001_ $$00009-0009-4039-4925$$aKnehr, Antje$$b6
000276320 7001_ $$00009-0009-0074-3019$$aStuart-Lopez, Geoffrey$$b7
000276320 7001_ $$00009-0009-3180-7847$$aVercruysse, Pauline$$b8
000276320 7001_ $$00009-0009-9951-3677$$aDieterlé, Stéphane$$b9
000276320 7001_ $$0P:(DE-2719)9001116$$aWeydt, Patrick$$b10
000276320 7001_ $$0P:(DE-2719)9001951$$aDorst, Johannes$$b11
000276320 7001_ $$00000-0002-0785-379X$$aKandler, Katharina$$b12
000276320 7001_ $$0P:(DE-2719)9001967$$aKassubek, Jan$$b13
000276320 7001_ $$00009-0004-4057-2975$$aWassermann, Laura$$b14
000276320 7001_ $$00000-0002-5724-2903$$aRouaux, Caroline$$b15
000276320 7001_ $$00000-0001-7271-5468$$aArthaud, Sébastien$$b16
000276320 7001_ $$00000-0003-1986-4565$$aDa Cruz, Sandrine$$b17
000276320 7001_ $$00000-0002-0503-423X$$aLuppi, Pierre-Hervé$$b18
000276320 7001_ $$0P:(DE-2719)2812851$$aRoselli, Francesco$$b19
000276320 7001_ $$0P:(DE-2719)2812633$$aLudolph, Albert C$$b20
000276320 7001_ $$00000-0002-0902-5824$$aDupuis, Luc$$b21
000276320 7001_ $$00000-0003-2313-2770$$aBolborea, Matei$$b22
000276320 773__ $$0PERI:(DE-600)2518839-2$$a10.1126/scitranslmed.adm7580$$gVol. 17, no. 783, p. eadm7580$$n783$$peadm7580$$tScience translational medicine$$v17$$x1946-6234$$y2025
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