Reduced number of hypocretin neurons in human narcolepsy.

Abstract:

:Murine and canine narcolepsy can be caused by mutations of the hypocretin (Hcrt) (orexin) precursor or Hcrt receptor genes. In contrast to these animal models, most human narcolepsy is not familial, is discordant in identical twins, and has not been linked to mutations of the Hcrt system. Thus, the cause of human narcolepsy remains unknown. Here we show that human narcoleptics have an 85%-95% reduction in the number of Hcrt neurons. Melanin-concentrating hormone (MCH) neurons, which are intermixed with Hcrt cells in the normal brain, are not reduced in number, indicating that cell loss is relatively specific for Hcrt neurons. The presence of gliosis in the hypocretin cell region is consistent with a degenerative process being the cause of the Hcrt cell loss in narcolepsy.

journal_name

Neuron

journal_title

Neuron

authors

Thannickal TC,Moore RY,Nienhuis R,Ramanathan L,Gulyani S,Aldrich M,Cornford M,Siegel JM

doi

10.1016/s0896-6273(00)00058-1

subject

Has Abstract

pub_date

2000-09-01 00:00:00

pages

469-74

issue

3

eissn

0896-6273

issn

1097-4199

pii

S0896-6273(00)00058-1

journal_volume

27

pub_type

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