{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T15:06:43Z","timestamp":1791558403862,"version":"4.3.4"},"reference-count":30,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,5,9]],"date-time":"2019-05-09T00:00:00Z","timestamp":1557360000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Bethlem myopathy is caused by dysfunctional collagen VI assembly, leading to varying degrees of hyperlaxity, contractures and muscle weakness. Previous studies demonstrate that cardiovascular training is safe and beneficial in patients with myopathies. However, exercise exacerbates the dystrophic phenotype in collagen VI\u2010knockout mice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Six men with Bethlem myopathy were included (4 training; 2 controls). After training, 2 patients detrained. Patients performed 10 weeks of home\u2010based, moderate\u2010intensity exercise monitored by a pulse\u2010watch. The primary outcome was change in peak oxygen uptake (VO<jats:sub>2peak<\/jats:sub>). Secondary outcomes were performances in functional tests.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>VO<jats:sub>2peak<\/jats:sub> improved in the training group (16%, <jats:italic>P<\/jats:italic> = 0.017). Detraining led to regression of VO<jats:sub>2peak<\/jats:sub> toward baseline values (\u20108%; <jats:italic>P<\/jats:italic> = 0.03). No change was seen in the control group (\u20107%; <jats:italic>P<\/jats:italic> = 0.47). Performance in functional tests did not change significantly. Creatine kinase values were stable during the study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Moderate\u2010intensity exercise seems to safely improve oxidative function in patients with Bethlem myopathy. <jats:italic>Muscle Nerve<\/jats:italic> <jats:bold>60<\/jats:bold>: 183\u2013188, 2019<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.26498","type":"journal-article","created":{"date-parts":[[2019,4,26]],"date-time":"2019-04-26T17:00:11Z","timestamp":1556298011000},"page":"183-188","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Moderate\u2010intensity aerobic exercise improves physical fitness in bethlem myopathy"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0834-206X","authenticated-orcid":false,"given":"Christoffer Rasmus","family":"Vissing","sequence":"first","affiliation":[{"name":"Copenhagen Neuromuscular Center, Department of Neurology University of Copenhagen  Rigshospitalet, Blegdamsvej 9, DK\u20102100 Copenhagen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gitte","family":"Hedermann","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center, Department of Neurology University of Copenhagen  Rigshospitalet, Blegdamsvej 9, DK\u20102100 Copenhagen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Vissing","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center, Department of Neurology University of Copenhagen  Rigshospitalet, Blegdamsvej 9, DK\u20102100 Copenhagen"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,5,9]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrneurol.2011.81"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0996-113"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1270"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/nm.2247"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.4161\/auto.7.12.17877"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddu254"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddp126"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1476-5381.2009.00316.x"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn289"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0800962105"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl149"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24112"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0146036"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20460"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000341274.61236.02"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000250358.32199.24"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn189"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-017-8497-9"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(99)00126-1"},{"key":"e_1_2_6_21_1","first-page":"45","article-title":"Strength and endurance in the therapeutic evaluation of prednisolone\u2010treated MDX mice","volume":"79","author":"Hudecki MS","year":"1993","journal-title":"Res Commun Chem Pathol Pharmacol"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.4172\/2329-9096.1000415"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.3233\/IES-2010-0389"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.joca.2013.05.002"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00194909"},{"key":"e_1_2_6_26_1","first-page":"170","article-title":"Mouse models of human disease","volume":"2016","author":"Perlman RL","year":"2016","journal-title":"Evol Med Public Health"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1989.03430170057028"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2016.05.014"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198811243192104"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.apmr.2004.09.012"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/jep.12629"}],"container-title":["Muscle &amp; 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