{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:46:52Z","timestamp":1786978012290,"version":"build-2736575974"},"reference-count":31,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,2,20]],"date-time":"2022-02-20T00:00:00Z","timestamp":1645315200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100013327","name":"H. Lundbeck A\/S","doi-asserted-by":"publisher","award":["R289\u20102018\u20101980"],"award-info":[{"award-number":["R289\u20102018\u20101980"]}],"id":[{"id":"10.13039\/501100013327","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Patients with glycogen storage disease type V (GSDV), also known as McArdle disease, have blocked glycogen breakdown due to myophosphorylase deficiency, leading to exercise intolerance, muscle pain, and risk of muscle damage. Blood\u2010derived ketone bodies (KBs) constitute an alternative energy source that could fuel the muscle independent of glycogenolysis. However, except for long\u2010time fasting or ketogenic dieting, KBs are present in low quantities. This led us to explore the effects of a drink containing exogenously produced KBs in the form of D\u2010\u03b2\u2010hydroxybutyrate esters (KE) on exercise capacity and metabolism in patients with GSDV. Eight GSDV patients and four healthy controls (HC) were included in this placebo\u2010controlled, cross\u2010over study where subjects were randomized to receive a KE drink with 395\u2009mgKE\/kg or placebo drink on two separate days 25 min before a submaximal cycle exercise test. The primary outcome was exercise capacity as indicated by heart rate response (HR) to exercise. Secondary outcomes included perceived exertion (PE) and measures of KB, carbohydrate, and fat metabolism during exercise. In GSDV, the KE drink vs. placebo increased plasma KBs and KB oxidation (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009\u2264\u20090.0001) but did not improve exercise capacity as judged from HR (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.120) and PE (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.109). In addition, the KE drink lowered plasma glucose, free fatty acids, and lowered lipolytic rate and glucose rate of appearance compared with placebo. Similar results were found in the HC group. The present study indicates that an increase in KB oxidation by oral KE supplementation does not improve exercise capacity in GSDV possibly because of KB\u2010induced inhibition of lipolysis and liver glucose output. Thus, oral KE supplementation alone cannot be recommended as a treatment option for patients with GSDV.\n                  <\/jats:p>","DOI":"10.1002\/jimd.12484","type":"journal-article","created":{"date-parts":[[2022,2,12]],"date-time":"2022-02-12T06:54:08Z","timestamp":1644648848000},"page":"502-516","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["No effect of oral ketone ester supplementation on exercise capacity in patients with\n                    <scp>McArdle<\/scp>\n                    disease and healthy controls: A randomized placebo\u2010controlled cross\u2010over study"],"prefix":"10.1002","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5494-8484","authenticated-orcid":false,"given":"Nicoline","family":"L\u00f8kken","sequence":"first","affiliation":[{"name":"Copenhagen Neuromuscular Center Copenhagen University Hospital \u2013 Rigshospitalet  Copenhagen Denmark"},{"name":"Department of Clinical Medicine University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jesper H.","family":"Storgaard","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center Copenhagen University Hospital \u2013 Rigshospitalet  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karoline L.","family":"Revsbech","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center Copenhagen University Hospital \u2013 Rigshospitalet  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicol C.","family":"Voermans","sequence":"additional","affiliation":[{"name":"The Department of Neurology, Donders Institute for Brain, Cognition and Behaviour Radboud University Nijmegen Medical Centre  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerrit","family":"Van Hall","sequence":"additional","affiliation":[{"name":"Clinical Metabolomics Core Facility, Clinical Biochemistry Copenhagen University Hospital  Copenhagen Denmark"},{"name":"Department of Biomedical Sciences, Faculty of Health &amp; Medical Sciences University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Vissing","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center Copenhagen University Hospital \u2013 Rigshospitalet  Copenhagen Denmark"},{"name":"Department of Clinical Medicine University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mette C.","family":"\u00d8rngreen","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center Copenhagen University Hospital \u2013 Rigshospitalet  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,2,20]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e328364dbdc"},{"issue":"1","key":"e_1_2_11_3_1","first-page":"35","article-title":"Muscle glycogenoses: an overview","volume":"26","author":"Di Mauro S","year":"2007","journal-title":"Acta Myol."},{"issue":"2","key":"e_1_2_11_4_1","first-page":"351","article-title":"State of the art in muscle lipid diseases","volume":"29","author":"Liang W\u2010C","year":"2010","journal-title":"Acta 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