Distinct narcolepsy syndromes in Orexin receptor-2 and Orexin null mice: molecular genetic dissection of Non-REM and REM sleep regulatory processes

Research output: Contribution to journalJournal articleResearchpeer-review

  • Jon T Willie
  • Richard M Chemelli
  • Christopher M Sinton
  • Shigeru Tokita
  • S Clay Williams
  • Yaz Y Kisanuki
  • Jacob N Marcus
  • Charlotte Lee
  • Joel K Elmquist
  • Kohlmeier, Kristi Anne
  • Christopher S Leonard
  • James A Richardson
  • Robert E Hammer
  • Masashi Yanagisawa
Narcolepsy-cataplexy, a neurological disorder associated with the absence of hypothalamic orexin (hypocretin) neuropeptides, consists of two underlying problems: inability to maintain wakefulness and intrusion of rapid eye movement (REM) sleep into wakefulness. Here we document, using behavioral, electrophysiological, and pharmacological criteria, two distinct classes of behavioral arrests exhibited by mice deficient in orexin-mediated signaling. Both OX2R(-/-) and orexin(-/-) mice are similarly affected with behaviorally abnormal attacks of non-REM sleep ("sleep attacks") and show similar degrees of disrupted wakefulness. In contrast, OX2R(-/-) mice are only mildly affected with cataplexy-like attacks of REM sleep, whereas orexin(-/-) mice are severely affected. Absence of OX2Rs eliminates orexin-evoked excitation of histaminergic neurons in the hypothalamus, which gate non-REM sleep onset. While normal regulation of wake/non-REM sleep transitions depends critically upon OX2R activation, the profound dysregulation of REM sleep control unique to the narcolepsy-cataplexy syndrome emerges from loss of signaling through both OX2R-dependent and OX2R-independent pathways.
Original languageEnglish
JournalNeuron
Volume38
Issue number5
Pages (from-to)715-30
Number of pages16
ISSN0896-6273
Publication statusPublished - 2003
Externally publishedYes

    Research areas

  • Animals, Arousal, Carrier Proteins, Cells, Cultured, Clomipramine, Disease Models, Animal, Efferent Pathways, Electroencephalography, Electromyography, Histamine, Hypothalamic Area, Lateral, Hypothalamus, Intracellular Signaling Peptides and Proteins, Male, Mice, Mice, Knockout, Narcolepsy, Neuropeptides, Receptors, G-Protein-Coupled, Receptors, Neuropeptide, Sleep, Sleep, REM, Synaptic Transmission

ID: 38346557