{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:26:01Z","timestamp":1786976761061,"version":"build-2736575974"},"reference-count":30,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,11,27]],"date-time":"2019-11-27T00:00:00Z","timestamp":1574812800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100006197","name":"A.P. M\u00f8ller Fonden til L\u00e6gevidenskabens Fremme","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006197","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003035","name":"Aase and Ejnar Danielsen Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003035","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000887","name":"Huntington's Disease Society of America","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000887","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009708","name":"Novo Nordisk Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100009708","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100010359","name":"Doctor Soren Segel and Johanne Wiibroe Segel\u2019s Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100010359","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>\n                      Huntington disease (HD) is an autosomal dominantly inherited neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin\n                      <jats:italic>(HTT)<\/jats:italic>\n                      gene. No disease\u2010modifying therapy exists for the treatment of patients with HD. The purpose of this study was therefore to investigate early disease mechanisms that potentially could be used as a target therapeutically.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Lymphocyte activity in cerebrospinal fluid (CSF) from 4 cohorts of\n                      <jats:italic>HTT<\/jats:italic>\n                      gene expansion carriers (n = 121 in total) and controls was analyzed by techniques based on flow cytometry and enzyme\u2010linked immunosorbent assays.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The data of this study provide evidence of immune abnormalities before motor onset of disease. In CSF of\n                      <jats:italic>HTT<\/jats:italic>\n                      gene expansion carriers, we found increased levels of proinflammatory cytokines, including IL\u201017, and increased consumption of the lymphocyte growth factor IL\u20107 before motor onset of HD. In concordance, we observed an increased prevalence of IL\u201017\u2013producing Th17.1 cells in the CSF of\n                      <jats:italic>HTT<\/jats:italic>\n                      gene expansion carriers, predominantly in pre\u2013motor manifest individuals. The frequency of intrathecal Th17.1 cells correlated negatively with progression of HD and the level of neurodegeneration, suggesting a role of Th17.1 cells in the early disease stage. We also observed a skewing in the balance between proinflammatory and regulatory T cells potentially favoring a proinflammatory intrathecal environment in\n                      <jats:italic>HTT<\/jats:italic>\n                      gene expansion carriers.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>\n                      These data suggest that Th17.1 cells are implicated in the earliest pathogenic phases of HD and suggest that treatment to dampen T \u2010cell\u2013driven inflammation before motor onset might be of benefit in\n                      <jats:italic>HTT<\/jats:italic>\n                      gene expansion carriers. ANN NEUROL 2020;87:246\u2013255\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ana.25647","type":"journal-article","created":{"date-parts":[[2019,11,14]],"date-time":"2019-11-14T12:30:58Z","timestamp":1573734658000},"page":"246-255","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":45,"title":["Early Intrathecal T Helper 17.1 Cell Activity in Huntington Disease"],"prefix":"10.1002","volume":"87","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9367-5020","authenticated-orcid":false,"given":"Marina R.","family":"von Essen","sequence":"first","affiliation":[{"name":"Danish Multiple Sclerosis Center, Department of Neurology, Rigshospitalet University of Copenhagen  Glostrup Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie N. N.","family":"Hellem","sequence":"additional","affiliation":[{"name":"Neurogenetics Clinic, Danish Dementia Research Center, Rigshospitalet University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tua","family":"Vinther\u2010Jensen","sequence":"additional","affiliation":[{"name":"Neurogenetics Clinic, Danish Dementia Research Center, Rigshospitalet University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilie","family":"Ammitzb\u00f8ll","sequence":"additional","affiliation":[{"name":"Danish Multiple Sclerosis Center, Department of Neurology, Rigshospitalet University of Copenhagen  Glostrup Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rikke H.","family":"Hansen","sequence":"additional","affiliation":[{"name":"Danish Multiple Sclerosis Center, Department of Neurology, Rigshospitalet University of Copenhagen  Glostrup 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