Abstract
Narcolepsy with cataplexy is a debilitating sleep disorder with an estimated prevalence of about 0.05%. Narcolepsy is caused by a selective loss of hypocretin (orexin) producing neurons in the perifornical hypothalamus. Based on the very strong association with the HLA subtype DQB1*0602, it is currently hypothesized narcolepsy is caused by an autoimmune-mediated process directed at the hypocretin neurons. So far however, studies focusing on general markers of (auto)immune activation, as well as humoral immunity against the hypocretin system have not yielded consistent results supporting this hypothesis.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Autoantibodies / immunology
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Genetic Linkage
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HLA-DQ Antigens / genetics
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HLA-DQ Antigens / immunology*
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HLA-DQ beta-Chains
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Humans
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Intracellular Signaling Peptides and Proteins / genetics
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Intracellular Signaling Peptides and Proteins / metabolism
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Membrane Glycoproteins / genetics
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Membrane Glycoproteins / immunology*
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Narcolepsy / genetics
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Narcolepsy / immunology*
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Narcolepsy / metabolism
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Neuropeptides / genetics
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Neuropeptides / metabolism
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Orexins
Substances
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Autoantibodies
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HLA-DQ Antigens
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HLA-DQ beta-Chains
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HLA-DQB1 antigen
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Intracellular Signaling Peptides and Proteins
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Membrane Glycoproteins
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Neuropeptides
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Orexins