{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,13]],"date-time":"2026-09-13T18:05:51Z","timestamp":1789322751734,"version":"build-2803163510"},"reference-count":22,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2021,9,13]],"date-time":"2021-09-13T00:00:00Z","timestamp":1631491200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100001962","name":"A-T Children's Project","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001962","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006209","name":"Radboud Universitair Medisch Centrum","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006209","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Treatment of animal models with ataxia telangiectasia (A\u2010T) with nicotinamide riboside (NR) improved their neurological outcome and survival.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The aim of this study is to investigate the effects of NR in patients with A\u2010T.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this open\u2010label, proof\u2010of\u2010concept study, 24 patients with A\u2010T were treated with NR during four consecutive months. The effects of NR on ataxia, dysarthria, quality of life, and laboratory parameters were analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>During treatment, ataxia scores improved; mean total Scale for the Assessment and Rating of Ataxia and International Cooperative Ataxia Rating Scale scores decreased to 2.4 and 10.1 points, respectively. After NR withdrawal, ataxia scores worsened. In immunodeficient patients, the mean serum IgG concentration increased substantially until the end of the study period with 0.52\u2009g\/L. Untargeted metabolomics analysis revealed increased plasma levels of NR metabolites and purine nucleosides during treatment. Adverse effects did not occur.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Treatment with NR is tolerated well and associated with improvement in ataxia and serum immunoglobulin concentrations in patients with A\u2010T. \u00a9 2021 The Authors.\n                      <jats:italic>Movement Disorders<\/jats:italic>\n                      published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mds.28788","type":"journal-article","created":{"date-parts":[[2021,9,13]],"date-time":"2021-09-13T06:06:02Z","timestamp":1631513162000},"page":"2951-2957","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":49,"title":["Nicotinamide Riboside Improves Ataxia Scores and Immunoglobulin Levels in Ataxia Telangiectasia"],"prefix":"10.1002","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1361-5920","authenticated-orcid":false,"given":"Stefanie J.G.","family":"Veenhuis","sequence":"first","affiliation":[{"name":"Department of Pediatrics Amalia Children's Hospital, Radboud University Medical Center  Nijmegen The Netherlands"},{"name":"Donders Institute for Brain, Cognition and Behavior Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nienke J.H.","family":"van Os","sequence":"additional","affiliation":[{"name":"Donders Institute for Brain, Cognition and Behavior Radboud University Medical Center  Nijmegen The Netherlands"},{"name":"Department of Neurology Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anjo J.W.M.","family":"Janssen","sequence":"additional","affiliation":[{"name":"Department of Rehabilitation Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marjo H.J.C.","family":"van Gerven","sequence":"additional","affiliation":[{"name":"Department of Rehabilitation Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karlien L.M.","family":"Coene","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Translation Metabolic Laboratory Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Udo. F.H.","family":"Engelke","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Translation Metabolic Laboratory Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ron A.","family":"Wevers","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Translation Metabolic Laboratory Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerjen H.","family":"Tinnevelt","sequence":"additional","affiliation":[{"name":"Institute for Molecules and Materials Radboud University  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rob","family":"ter Heine","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Radboud University Medical Center  Nijmegen The Netherlands"},{"name":"Radboud Institute for Health Sciences Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bart P.C.","family":"van de Warrenburg","sequence":"additional","affiliation":[{"name":"Donders Institute for Brain, Cognition and Behavior Radboud University Medical Center  Nijmegen The Netherlands"},{"name":"Department of Neurology Radboud University Medical Center  Nijmegen The Netherlands"},{"name":"Center of Expertise for Parkinson &amp; 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