The immunopathogenesis of narcolepsy type 1
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The immunopathogenesis of narcolepsy type 1. / Liblau, Roland S.; Latorre, Daniela; Kornum, Birgitte R.; Dauvilliers, Yves; Mignot, Emmanuel J.
In: Nature Reviews Immunology, Vol. 24, 2024, p. 33-48.Research output: Contribution to journal › Review › Research › peer-review
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TY - JOUR
T1 - The immunopathogenesis of narcolepsy type 1
AU - Liblau, Roland S.
AU - Latorre, Daniela
AU - Kornum, Birgitte R.
AU - Dauvilliers, Yves
AU - Mignot, Emmanuel J.
N1 - Publisher Copyright: © 2023, Springer Nature Limited.
PY - 2024
Y1 - 2024
N2 - Narcolepsy type 1 (NT1) is a chronic sleep disorder resulting from the loss of a small population of hypothalamic neurons that produce wake-promoting hypocretin (HCRT; also known as orexin) peptides. An immune-mediated pathology for NT1 has long been suspected given its exceptionally tight association with the MHC class II allele HLA-DQB1*06:02, as well as recent genetic evidence showing associations with polymorphisms of T cell receptor genes and other immune-relevant loci and the increased incidence of NT1 that has been observed after vaccination with the influenza vaccine Pandemrix. The search for both self-antigens and foreign antigens recognized by the pathogenic T cell response in NT1 is ongoing. Increased T cell reactivity against HCRT has been consistently reported in patients with NT1, but data demonstrating a primary role for T cells in neuronal destruction are currently lacking. Animal models are providing clues regarding the roles of autoreactive CD4+ and CD8+ T cells in the disease. Elucidation of the pathogenesis of NT1 will allow for the development of targeted immunotherapies at disease onset and could serve as a model for other immune-mediated neurological diseases.
AB - Narcolepsy type 1 (NT1) is a chronic sleep disorder resulting from the loss of a small population of hypothalamic neurons that produce wake-promoting hypocretin (HCRT; also known as orexin) peptides. An immune-mediated pathology for NT1 has long been suspected given its exceptionally tight association with the MHC class II allele HLA-DQB1*06:02, as well as recent genetic evidence showing associations with polymorphisms of T cell receptor genes and other immune-relevant loci and the increased incidence of NT1 that has been observed after vaccination with the influenza vaccine Pandemrix. The search for both self-antigens and foreign antigens recognized by the pathogenic T cell response in NT1 is ongoing. Increased T cell reactivity against HCRT has been consistently reported in patients with NT1, but data demonstrating a primary role for T cells in neuronal destruction are currently lacking. Animal models are providing clues regarding the roles of autoreactive CD4+ and CD8+ T cells in the disease. Elucidation of the pathogenesis of NT1 will allow for the development of targeted immunotherapies at disease onset and could serve as a model for other immune-mediated neurological diseases.
U2 - 10.1038/s41577-023-00902-9
DO - 10.1038/s41577-023-00902-9
M3 - Review
C2 - 37400646
AN - SCOPUS:85163727183
VL - 24
SP - 33
EP - 48
JO - Nature Reviews Immunology
JF - Nature Reviews Immunology
SN - 1474-1733
ER -
ID: 360030070