{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T06:25:07Z","timestamp":1789712707925,"version":"4.0.1"},"reference-count":37,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2014,8,13]],"date-time":"2014-08-13T00:00:00Z","timestamp":1407888000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008368","name":"Gigtforeningen","doi-asserted-by":"publisher","award":["R 108\u2010A2458"],"award-info":[{"award-number":["R 108\u2010A2458"]}],"id":[{"id":"10.13039\/100008368","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004385","name":"Universiteit Gent","doi-asserted-by":"publisher","award":["08\/01M01108"],"award-info":[{"award-number":["08\/01M01108"]}],"id":[{"id":"10.13039\/501100004385","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2014,11]]},"abstract":"<jats:p>\n                    There is a clinical overlap between classic Ehlers\u2010Danlos syndrome (cEDS) and benign joint hypermobility syndrome (BJHS), with hypermobility as the main symptom. The purpose of this study was to investigate the role of type V collagen mutations and tendon pathology in these 2 syndromes. In patients (cEDS,\n                    <jats:italic>n<\/jats:italic>\n                    =7; BJHS,\n                    <jats:italic>n<\/jats:italic>\n                    =8) and controls (Ctrl,\n                    <jats:italic>n<\/jats:italic>\n                    =8), we measured patellar tendon ultrastructure (transmission electron microscopy), dimensions (magnetic resonance imaging), and biomechanical properties (force and ultrasonographic measurements during a ramped isometric knee extension). Mutation analyses (\n                    <jats:italic>COL5A1<\/jats:italic>\n                    and\n                    <jats:italic>COL5A2<\/jats:italic>\n                    ) were performed in the patients.\n                    <jats:italic>COL5A1<\/jats:italic>\n                    mutations were found in 3 of 4 of the patients with cEDS. Patellar tendon dimensions were similar between the groups, but large, irregular collagen fibrils were in 4 of 5 patients with cEDS. In the cEDS group, tendon stiffness and Young's modulus were reduced to ~50% of that in BJHS and Ctrl groups (\n                    <jats:italic>P<\/jats:italic>\n                    &lt;0.05). The nonhypermobile, healthy controls were matched with the patients in age, sex, body weight, and physical activity, to compare outcomes.\n                    <jats:italic>COL5A1<\/jats:italic>\n                    mutations led to structural tendon pathology and low tendon stiffness in cEDS, explaining the patients' hypermobility, whereas no tendon pathology was found that explained the hypermobility in BJHS.\u2014Nielsen, R. H., Coupp\u00e9, C., Jensen, J. K., Olsen, M. R., Heinemeier, K. M., Malfait, F., Symoens, S., De Paepe, A., Schjerling, P., Magnusson, S. P., Remvig, L., Kjaer, M., Low tendon stiffness and abnormal ultrastructure distinguish classic Ehlers\u2010Danlos syndrome from benign joint hypermobility syndrome in patients. FASEB J. 28, 4668\u20134676 (2014).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.14-249656","type":"journal-article","created":{"date-parts":[[2014,8,14]],"date-time":"2014-08-14T00:11:25Z","timestamp":1407975085000},"page":"4668-4676","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":43,"title":["Low tendon stiffness and abnormal ultrastructure distinguish classic Ehlers\u2010Danlos syndrome from benign joint hypermobility syndrome in patients"],"prefix":"10.1096","volume":"28","author":[{"given":"Rie Harboe","family":"Nielsen","sequence":"first","affiliation":[{"name":"Institute of Sports Medicine Department of Orthopaedic Surgery Bispebjerg Hospital Copenhagen Denmark"},{"name":"Faculty of Health and Medical Sciences University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Coupp\u00e9","sequence":"additional","affiliation":[{"name":"Musculoskeletal Rehabilitation Research Unit Department of Physical Therapy Bispebjerg Hospital Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacob Kildevang","family":"Jensen","sequence":"additional","affiliation":[{"name":"Institute of Sports Medicine Department of Orthopaedic Surgery Bispebjerg Hospital Copenhagen Denmark"},{"name":"Faculty of Health and Medical Sciences University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morten Raun","family":"Olsen","sequence":"additional","affiliation":[{"name":"Institute of Sports Medicine Department of Orthopaedic Surgery Bispebjerg Hospital Copenhagen Denmark"},{"name":"Faculty of Health and Medical Sciences University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katja Maria","family":"Heinemeier","sequence":"additional","affiliation":[{"name":"Institute of Sports Medicine Department of Orthopaedic Surgery Bispebjerg Hospital Copenhagen Denmark"},{"name":"Faculty of Health and Medical Sciences University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fransiska","family":"Malfait","sequence":"additional","affiliation":[{"name":"Centre for Medical Genetics University Hospital Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofie SymoensAnne","family":"De Paepe","sequence":"additional","affiliation":[{"name":"Centre for Medical Genetics University Hospital Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Schjerling","sequence":"additional","affiliation":[{"name":"Institute of Sports Medicine Department of Orthopaedic Surgery Bispebjerg Hospital Copenhagen Denmark"},{"name":"Faculty of Health and Medical Sciences University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stig Peter","family":"Magnusson","sequence":"additional","affiliation":[{"name":"Musculoskeletal Rehabilitation Research Unit Department of Physical Therapy Bispebjerg Hospital Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Remvig","sequence":"additional","affiliation":[{"name":"Department of Rheumatology Rigshospitalet Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Kjaer","sequence":"additional","affiliation":[{"name":"Institute of Sports Medicine Department of Orthopaedic Surgery Bispebjerg Hospital Copenhagen Denmark"},{"name":"Faculty of Health and Medical Sciences University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,8,13]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO;2-O"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0049-0172(72)90004-2"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22137"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.5402\/2012\/751768"},{"key":"e_1_2_7_6_1","first-page":"1777","article-title":"The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS)","volume":"27","author":"Grahame R.","year":"2000","journal-title":"J. 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