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Curr Opin Neurobiol. 2011 Sep 30. [Epub ahead of print]
Narcolepsy with hypocretin/orexin deficiency, infections and autoimmunity of the brain.
Kornum BR, Faraco J, Mignot E.
Source
Stanford Center for Sleep Sciences and Medicine, Stanford University School of Medicine, 1050A Arastradero Rd., Palo Alto, CA 94304, USA.
Abstract
The loss of hypothalamic hypocretin/orexin (hcrt) producing neurons causes narcolepsy with cataplexy. An autoimmune basis for the disease has long been suspected and recent results have greatly strengthened this hypothesis. Narcolepsy with hcrt deficiency is now known to be associated with a Human Leukocyte Antigen (HLA) and T-cell receptor (TCR) polymorphisms, suggesting that an autoimmune process targets a single peptide unique to hcrt-cells via specific HLA-peptide-TCR interactions. Recent data have shown a robust seasonality of disease onset in children and associations with Streptococcus Pyogenes, and influenza A H1N1-infection and H1N1-vaccination, pointing towards processes such as molecular mimicry or bystander activation as crucial for disease development. We speculate that upper airway infections may be common precipitants of a whole host of CNS autoimmune complications including narcolepsy.
Copyright ? 2011 Elsevier Ltd. All rights reserved.
PMID:
21963829
[PubMed - as supplied by publisher]
Narcolepsy with hypocretin/orexin deficiency, infections and autoimmunity of the brain.
Kornum BR, Faraco J, Mignot E.
Source
Stanford Center for Sleep Sciences and Medicine, Stanford University School of Medicine, 1050A Arastradero Rd., Palo Alto, CA 94304, USA.
Abstract
The loss of hypothalamic hypocretin/orexin (hcrt) producing neurons causes narcolepsy with cataplexy. An autoimmune basis for the disease has long been suspected and recent results have greatly strengthened this hypothesis. Narcolepsy with hcrt deficiency is now known to be associated with a Human Leukocyte Antigen (HLA) and T-cell receptor (TCR) polymorphisms, suggesting that an autoimmune process targets a single peptide unique to hcrt-cells via specific HLA-peptide-TCR interactions. Recent data have shown a robust seasonality of disease onset in children and associations with Streptococcus Pyogenes, and influenza A H1N1-infection and H1N1-vaccination, pointing towards processes such as molecular mimicry or bystander activation as crucial for disease development. We speculate that upper airway infections may be common precipitants of a whole host of CNS autoimmune complications including narcolepsy.
Copyright ? 2011 Elsevier Ltd. All rights reserved.
PMID:
21963829
[PubMed - as supplied by publisher]
