{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T12:30:51Z","timestamp":1791117051062,"version":"4.1.0"},"reference-count":25,"publisher":"National Academy of Sciences","issue":"2","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2010,1,12]]},"abstract":"<jats:p>An aneurysm of the aorta is a common pathology characterized by segmental weakening of the artery. Although it is generally accepted that the vessel-wall weakening is caused by an impaired collagen metabolism, a clear association has been demonstrated only for rare syndromes such as the vascular type Ehlers\u2013Danlos syndrome. Here we show that vessel-wall failure in growing aneurysms of patients who have aortic abdominal aneurysm (AAA) or Marfan syndrome is not related to a collagen defect at the molecular level. On the contrary our findings indicate similar (Marfan) or even higher collagen concentrations (AAA) and increased collagen cross-linking in the aneurysms. Using 3D confocal imaging we show that the two conditions are associated with profound defects in collagen microarchitecture. Reconstructions of normal vessel wall show that adventitial collagen fibers are organized in a loose braiding of collagen ribbons. These ribbons encage the vessel, allowing the vessel to dilate easily but preventing overstretching. AAA and aneurysms in Marfan syndrome show dramatically altered collagen architectures with loss of the collagen knitting. Evaluations of the functional characteristics by atomic force microscopy showed that the wall has lost its ability to stretch easily and revealed a second defect: although vascular collagen in normal aortic wall behaves as a coherent network, in AAA and Marfan tissues it does not. As result, mechanical forces loaded on individual fibers are not distributed over the tissue. These studies demonstrate that the mechanical properties of tissue are strongly influenced by collagen microarchitecture and that perturbations in the collagen networks may lead to mechanical failure.<\/jats:p>","DOI":"10.1073\/pnas.0910312107","type":"journal-article","created":{"date-parts":[[2009,12,28]],"date-time":"2009-12-28T21:10:18Z","timestamp":1262034618000},"page":"862-865","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":179,"title":["Distinct defects in collagen microarchitecture underlie vessel-wall failure in advanced abdominal aneurysms and aneurysms in Marfan syndrome"],"prefix":"10.1073","volume":"107","author":[{"given":"Jan H. N.","family":"Lindeman","sequence":"first","affiliation":[{"name":"Departments of aVascular Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian A.","family":"Ashcroft","sequence":"additional","affiliation":[{"name":"Leiden Institute of Physics, Leiden University, 2300 RA Leiden, The Netherlands; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan-Willem M.","family":"Beenakker","sequence":"additional","affiliation":[{"name":"Leiden Institute of Physics, Leiden University, 2300 RA Leiden, The Netherlands; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maarten","family":"van Es","sequence":"additional","affiliation":[{"name":"Leiden Institute of Physics, Leiden University, 2300 RA Leiden, The Netherlands; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nico B. R.","family":"Koekkoek","sequence":"additional","affiliation":[{"name":"Leiden Institute of Physics, Leiden University, 2300 RA Leiden, The Netherlands; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frans A.","family":"Prins","sequence":"additional","affiliation":[{"name":"Pathology, Leiden University Medical Center, 2300 RC Leiden, The Netherlands;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jarl F.","family":"Tielemans","sequence":"additional","affiliation":[{"name":"Departments of aVascular Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hazem","family":"Abdul-Hussien","sequence":"additional","affiliation":[{"name":"Departments of aVascular Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruud A.","family":"Bank","sequence":"additional","affiliation":[{"name":"TNO-Quality of Life, BioSciences, 2333 CK Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tjerk H.","family":"Oosterkamp","sequence":"additional","affiliation":[{"name":"Leiden Institute of Physics, Leiden University, 2300 RA Leiden, The Netherlands; and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2009,12,28]]},"reference":[{"key":"e_1_3_2_1_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)66459-8"},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1067\/msg.2002.121421"},{"key":"e_1_3_2_3_2","first-page":"370","article-title":"Aneurysm or occlusive disease\u2014factors determining the clinical course of atherosclerosis of the infrarenal aorta","volume":"110","author":"McGee GS","year":"1991","unstructured":"GS McGee, et al., Aneurysm or occlusive disease\u2014factors determining the clinical course of atherosclerosis of the infrarenal aorta. Surgery 110, 370\u2013375, discussion 375\u2013376. (1991).","journal-title":"Surgery"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/0741-5214(89)90409-6"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/0741-5214(87)90274-6"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M709319200"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/jcb.10413"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2818.2001.00832.x"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1001\/archsurg.1984.01390160041009"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9610(79)90370-2"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00424-007-0398-9"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/nmat1428"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1080\/10520290600783143"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1115\/1.2898830"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.0000174129.77391.55"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0741-5214(94)90169-4"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cpcardiol.2007.10.001"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1520-6777(1998)17:2<135::AID-NAU7>3.0.CO;2-E"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1242\/jeb.202.23.3305"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0950-821X(05)80597-3"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2006.046250"},{"key":"e_1_3_2_22_2","first-page":"87","article-title":"Study of fibroblasts in Marfan\u2019s syndrome","volume":"3","author":"Macek M","year":"1966","unstructured":"M Macek, J Hurych, M Chvapil, V Kadlecov\u00e1, Study of fibroblasts in Marfan\u2019s syndrome. Humangenetik 3, 87\u201397 (1966).","journal-title":"Humangenetik"},{"key":"e_1_3_2_23_2","first-page":"271","volume-title":"Textbook of Surgery","author":"Madden JW","year":"1977","unstructured":"JW Madden, Wound healing. Textbook of Surgery, ed DC Sabiston (WB Saunders, Philadelphia), pp. 271\u2013294 (1977)."},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1097\/00006534-199104000-00032"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO;2-R"}],"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.0910312107","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,12]],"date-time":"2022-04-12T19:17:05Z","timestamp":1649791025000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.0910312107"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,12,28]]},"references-count":25,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2010,1,12]]}},"alternative-id":["10.1073\/pnas.0910312107"],"URL":"https:\/\/doi.org\/10.1073\/pnas.0910312107","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,12,28]]},"assertion":[{"value":"2009-12-28","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}