{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T05:11:01Z","timestamp":1788757861138,"version":"build-2803163510"},"reference-count":75,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T00:00:00Z","timestamp":1788652800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T00:00:00Z","timestamp":1788652800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["WA 1511\/3\u20101"],"award-info":[{"award-number":["WA 1511\/3\u20101"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["advanced.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Advanced Science"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Fibrillin\u20111 (\n                    <jats:italic>FBN1<\/jats:italic>\n                    ) mutations lead to extracellular matrix (ECM) defects with progressive aortic dilation in Marfan's syndrome (MFS). MFS thoracic aortas have increased stiffness, but how visco\u2010elastic and microstructural abnormalities affect in vivo hemodynamics remains inconclusive. We applied ex vivo uniaxial visco\u2010elasticity stress\u2010relaxation testing and simultaneous 3D collagen\n                    <jats:italic>Second Harmonic Generation<\/jats:italic>\n                    (SHG) imaging to MFS and wt littermate linearized aortic strips with in vivo vascular function. MFS mouse (\n                    <jats:italic>Fbn1<\/jats:italic>\n                    <jats:sup>mgR\/mgR<\/jats:sup>\n                    ) aortas were stiffer and more viscous. SHG imaging revealed MFS ECM disorganization at rest and stronger collagen\u2010alignment strain response than wt strips, indicating greater fibrillar straightening capacity. in vivo, MFS mice developed early, progressive aortic dilation. Pulse wave velocity was elevated in young MFS mice pre\u2010dilation but declined as aneurysms formed. Female MFS aortic peak pressure and velocity were normal, but reduced in MFS\u2010males vs. wt. Ex vivo perfused wt carotid arteries were highly compliant. Female MFS carotids were stiffer, had reduced radial strain, a right\u2011shifted pressure\u2010strain curve and minimal wall\u2011thickness changes. MFS carotids did not recover baseline diameter after perfusion, indicating impaired visco\u2010elastic recoil. We demonstrate early visco\u2010elastic MFS aortic dysfunction and ECM disorder. Mechanistically, the larger collagen\u2010straightening capacity in MFS cannot compensate for the increased visco\u2010elasticity during a beat\u2010to\u2010beat cycle.\n                  <\/jats:p>","DOI":"10.1002\/advs.77549","type":"journal-article","created":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T04:21:16Z","timestamp":1788754876000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Increased Disarray of Extracellular Matrix Collagen\u2010I Fiber Network and Compromised Biomechanics in Aortae From Marfan\u2010Syndrome Mice Assessed Through Combined Opto\u2010Biomechatronics"],"prefix":"10.1002","author":[{"given":"Dominik","family":"Schneidereit","sequence":"first","affiliation":[{"name":"Institute of Medical Biotechnology Department of Chemical and Biological Engineering Friedrich\u2010Alexander University Erlangen\u2010N\u00fcrnberg  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anika","family":"Nagel","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Pathophysiology Ruprecht\u2010Karls University Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-8480-3911","authenticated-orcid":false,"given":"Vanessa I. T.","family":"Zwaans","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Pathophysiology Ruprecht\u2010Karls University Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laureen","family":"Schurr","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology Department of Chemical and Biological Engineering Friedrich\u2010Alexander University Erlangen\u2010N\u00fcrnberg  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonas","family":"Ahr","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology Department of Chemical and Biological Engineering Friedrich\u2010Alexander University Erlangen\u2010N\u00fcrnberg  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5968-2593","authenticated-orcid":false,"given":"Michael","family":"Haug","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology Department of Chemical and Biological Engineering Friedrich\u2010Alexander University Erlangen\u2010N\u00fcrnberg  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Piluso","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Pathophysiology Ruprecht\u2010Karls University Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Sporkova","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Pathophysiology Ruprecht\u2010Karls University Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Karck","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery University Hospital Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2723-5953","authenticated-orcid":false,"given":"Andreas H.","family":"Wagner","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Pathophysiology Ruprecht\u2010Karls University Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2238-2049","authenticated-orcid":false,"given":"Oliver","family":"Friedrich","sequence":"additional","affiliation":[{"name":"Institute of Medical Biotechnology Department of Chemical and Biological Engineering Friedrich\u2010Alexander University Erlangen\u2010N\u00fcrnberg  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,6]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140\u20106736(05)67789\u20106"},{"key":"e_1_2_11_3_1","article-title":"Associations of Age and Sex with Marfan Phenotype: The National Heart, Lung, and Blood Institute GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) Registry","volume":"10","author":"Roman M. J.","year":"2017","journal-title":"Circulation: Cardiovascular Genetics"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21372"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2005.039669"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.7.3819"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200608167"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M803373200"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.202100282R"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.2840"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cellsig.2021.110104"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598\u2010017\u201001620\u20108"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.actbio.2025.05.056"},{"key":"e_1_2_11_14_1","first-page":"645","article-title":"Aortic Aneurysm in Marfan's Syndrome: Changes in the Ultrastructure and Composition of Collagen","volume":"129","author":"Scheck M.","year":"1979","journal-title":"Journal of Anatomy"},{"key":"e_1_2_11_15_1","first-page":"247","article-title":"Consensus Statement on Surgical Pathology of the Aorta from the Society for Cardiovascular Pathology and the Association For European Cardiovascular Pathology: II","volume":"25","author":"Halushka M. K.","year":"2016","journal-title":"Noninflammatory degenerative diseases\u2014nomenclature and diagnostic criteria"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00255.2021"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1046\u20102023(02)00292\u2010X"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2013.06.054"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41377\u2010018\u20100080\u20103"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jsb.2014.07.003"},{"key":"e_1_2_11_21_1","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1016\/j.biomaterials.2004.02.059","article-title":"Multiphoton Autofluorescence Imaging of Intratissue Elastic Fibers","volume":"26","author":"K\u00f6nig K.","year":"2005","journal-title":"Biomaterials"},{"key":"e_1_2_11_22_1","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-025-09665-w","article-title":"Exploring Thoracic Aorta ECM Alterations in Marfan Syndrome: Insights into Aorta Wall Structure","volume":"15","author":"Souza R. B.","year":"2025","journal-title":"Scientific Reports"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10439\u2010013\u20100799\u20101"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.3389\/fcvm.2021.800730"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mad.2021.111471"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmbbm.2021.104395"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.actbio.2021.05.025"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2021.785138"},{"key":"e_1_2_11_29_1","article-title":"Interplay between Viscoelasticity and Stiffness as Essential Properties in Mechanobiology","volume":"10","author":"Pajic\u2010Lijakovic I.","year":"2025","journal-title":"Advances in Physics: X"},{"key":"e_1_2_11_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcp.2022.115280"},{"key":"e_1_2_11_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcp.2019.03.034"},{"key":"e_1_2_11_32_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018\u2010016\u20102324\u20109"},{"key":"e_1_2_11_33_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl\u20102025\u2010327408"},{"key":"e_1_2_11_34_1","doi-asserted-by":"crossref","first-page":"1628","DOI":"10.1016\/j.jvs.2012.10.007","article-title":"The Fibrillin\u20101 Hypomorphic mgR\/mgR Murine Model of Marfan Syndrome Shows Severe Elastolysis in all Segments of the Aorta","volume":"57","author":"Schwill S.","year":"2013","journal-title":"Journal of Vascular Surgery"},{"key":"e_1_2_11_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.omtn.2017.08.014"},{"key":"e_1_2_11_36_1","doi-asserted-by":"crossref","first-page":"2364","DOI":"10.3390\/cancers14102364","article-title":"Multiphoton Microscopy Reveals DAPK1\u2010Dependent Extracellular Matrix Remodeling in a Chorioallantoic Membrane (CAM) Model","volume":"14","author":"Kunze P.","year":"2022","journal-title":"Cancers"},{"key":"e_1_2_11_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006\u20103495(02)75673\u20107"},{"key":"e_1_2_11_38_1","doi-asserted-by":"publisher","DOI":"10.1364\/OPTICA.4.001171"},{"key":"e_1_2_11_39_1","doi-asserted-by":"publisher","DOI":"10.1529\/biophysj.104.047308"},{"key":"e_1_2_11_40_1","doi-asserted-by":"publisher","DOI":"10.1364\/OE.15.012286"},{"key":"e_1_2_11_41_1","doi-asserted-by":"publisher","DOI":"10.1529\/biophysj.106.099457"},{"key":"e_1_2_11_42_1","doi-asserted-by":"publisher","DOI":"10.3791\/1291"},{"key":"e_1_2_11_43_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10439\u2010005\u20108478\u20105"},{"key":"e_1_2_11_44_1","doi-asserted-by":"publisher","DOI":"10.1186\/1480\u20109222\u201016\u20106"},{"key":"e_1_2_11_45_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022\u20105223(03)00728\u20101"},{"key":"e_1_2_11_46_1","doi-asserted-by":"publisher","DOI":"10.1093\/cvr\/cvag114"},{"key":"e_1_2_11_47_1","doi-asserted-by":"publisher","DOI":"10.1098\/rsif.2016.1036"},{"key":"e_1_2_11_48_1","doi-asserted-by":"publisher","DOI":"10.1113\/EP092672"},{"key":"e_1_2_11_49_1","doi-asserted-by":"publisher","DOI":"10.15420\/ecr\/2016:19:2"},{"key":"e_1_2_11_50_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmbbm.2025.106906"},{"key":"e_1_2_11_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmbbm.2023.106028"},{"key":"e_1_2_11_52_1","doi-asserted-by":"publisher","DOI":"10.1115\/1.1824130"},{"key":"e_1_2_11_53_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1431927615015585"},{"key":"e_1_2_11_54_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12265\u2010012\u20109349\u20108"},{"key":"e_1_2_11_55_1","doi-asserted-by":"publisher","DOI":"10.1098\/rsif.2012.1004"},{"key":"e_1_2_11_56_1","doi-asserted-by":"publisher","DOI":"10.21037\/acs.2017.09.12"},{"key":"e_1_2_11_57_1","first-page":"247","article-title":"Experimental\/Numerical Study to Assess Mechanical Properties of Healthy and Marfan Syndrome Ascending Thoracic Aorta under Axial and Circumferential Loading","volume":"31","author":"Jarrahi A.","year":"2016","journal-title":"Materials Technology"},{"key":"e_1_2_11_58_1","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezaf293"},{"key":"e_1_2_11_59_1","doi-asserted-by":"publisher","DOI":"10.1103\/PhysRevLett.125.068101"},{"key":"e_1_2_11_60_1","doi-asserted-by":"publisher","DOI":"10.1161\/HYPERTENSIONAHA.107.103440"},{"key":"e_1_2_11_61_1","doi-asserted-by":"publisher","DOI":"10.1038\/srep11064"},{"key":"e_1_2_11_62_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598\u2010021\u201002196\u20100"},{"key":"e_1_2_11_63_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10237\u2010024\u201001881\u2010z"},{"key":"e_1_2_11_64_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbiomech.2014.06.025"},{"key":"e_1_2_11_65_1","doi-asserted-by":"publisher","DOI":"10.1161\/HYPERTENSIONAHA.110.150110"},{"key":"e_1_2_11_66_1","doi-asserted-by":"publisher","DOI":"10.1038\/hr.2014.101"},{"key":"e_1_2_11_67_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.artres.2018.10.037"},{"key":"e_1_2_11_68_1","doi-asserted-by":"publisher","DOI":"10.1111\/eci.13885"},{"key":"e_1_2_11_69_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms241713571"},{"key":"e_1_2_11_70_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00262.2020"},{"key":"e_1_2_11_71_1","doi-asserted-by":"publisher","DOI":"10.1093\/gerona\/glag075"},{"key":"e_1_2_11_72_1","doi-asserted-by":"publisher","DOI":"10.1097\/HJH.0000000000001512"},{"key":"e_1_2_11_73_1","doi-asserted-by":"publisher","DOI":"10.3389\/fbioe.2025.1556091"},{"key":"e_1_2_11_74_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13073\u2010024\u201001423\u20103"},{"key":"e_1_2_11_75_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbiomech.2011.12.013"},{"key":"e_1_2_11_76_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.114.304936"}],"container-title":["Advanced Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/advanced.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/advs.77549","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/advanced.onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/advs.77549","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/advanced.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/advs.77549","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T04:21:18Z","timestamp":1788754878000},"score":1,"resource":{"primary":{"URL":"https:\/\/advanced.onlinelibrary.wiley.com\/doi\/10.1002\/advs.77549"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,6]]},"references-count":75,"alternative-id":["10.1002\/advs.77549"],"URL":"https:\/\/doi.org\/10.1002\/advs.77549","archive":["Portico"],"relation":{},"ISSN":["2198-3844","2198-3844"],"issn-type":[{"value":"2198-3844","type":"print"},{"value":"2198-3844","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,6]]},"assertion":[{"value":"2026-04-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-22","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-06","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e77549"}}