Print Email Facebook Twitter Distinct defects in collagen microarchitecture underlie vessel-wall failure in advanced abdominal aneurysms and aneurysms in Marfan syndrome Title Distinct defects in collagen microarchitecture underlie vessel-wall failure in advanced abdominal aneurysms and aneurysms in Marfan syndrome Author Lindeman, J.H.N. Ashcroft, B.A. Beenakker, J.-W.M. van Es, M. Koekkoek, N.B.R. Prins, F.A. Tielemans, J.F. Abdul-Hussien, H. Bank, R.A. Oosterkamp, T.H. TNO Kwaliteit van Leven Publication year 2010 Abstract An aneurysm of the aorta is a common pathology characterized by segmentalweakeningof the artery.Althoughit isgenerally 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-Danlos 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 vesselwall showthat 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 aorticwall behaves as a coherent network, inAAAand 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. Subject BiologyBiomedical Researchcollagenabdominal aorta aneurysmadultagedarticleatomic force microscopyclinical articlecollagen defectcontrolled studyhumanhuman tissueMarfan syndromepriority journalprotein analysisprotein structureAgedAorta, AbdominalAortic Aneurysm, AbdominalArteriesCollagenHumansHydroxyprolineMarfan SyndromeMicroscopy, ConfocalMiddle AgedProline To reference this document use: http://resolver.tudelft.nl/uuid:4b6c4d20-28d0-4dfa-8aac-094365d5fe57 TNO identifier 347467 ISSN 0027-8424 Source Proceedings of the National Academy of Sciences of the United States of America, 107 (2), 862-865 Document type article Files To receive the publication files, please send an e-mail request to TNO Library.