{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T04:49:19Z","timestamp":1788238159496,"version":"build-2803163510"},"reference-count":59,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2023,4,20]],"date-time":"2023-04-20T00:00:00Z","timestamp":1681948800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Hungarian National Research, Development and Innovation Office","award":["K135360"],"award-info":[{"award-number":["K135360"]}]},{"name":"Hungarian National Research, Development and Innovation Office","award":["TKP2021-EGA-23"],"award-info":[{"award-number":["TKP2021-EGA-23"]}]},{"name":"Hungarian National Research, Development and Innovation Office","award":["\u00daNKP-22-3-I-SE-49"],"award-info":[{"award-number":["\u00daNKP-22-3-I-SE-49"]}]},{"name":"Hungarian National Research, Development and Innovation Office","award":["RRF-2.3.1-21-2022-00003"],"award-info":[{"award-number":["RRF-2.3.1-21-2022-00003"]}]},{"name":"New National Excellence Program of the Ministry for Culture and Innovation from the source of the National Research, Development, and Innovation Fund","award":["K135360"],"award-info":[{"award-number":["K135360"]}]},{"name":"New National Excellence Program of the Ministry for Culture and Innovation from the source of the National Research, Development, and Innovation Fund","award":["TKP2021-EGA-23"],"award-info":[{"award-number":["TKP2021-EGA-23"]}]},{"name":"New National Excellence Program of the Ministry for Culture and Innovation from the source of the National Research, Development, and Innovation Fund","award":["\u00daNKP-22-3-I-SE-49"],"award-info":[{"award-number":["\u00daNKP-22-3-I-SE-49"]}]},{"name":"New National Excellence Program of the Ministry for Culture and Innovation from the source of the National Research, Development, and Innovation Fund","award":["RRF-2.3.1-21-2022-00003"],"award-info":[{"award-number":["RRF-2.3.1-21-2022-00003"]}]},{"DOI":"10.13039\/501100000780","name":"European Union","doi-asserted-by":"publisher","award":["K135360"],"award-info":[{"award-number":["K135360"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000780","name":"European Union","doi-asserted-by":"publisher","award":["TKP2021-EGA-23"],"award-info":[{"award-number":["TKP2021-EGA-23"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000780","name":"European Union","doi-asserted-by":"publisher","award":["\u00daNKP-22-3-I-SE-49"],"award-info":[{"award-number":["\u00daNKP-22-3-I-SE-49"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000780","name":"European Union","doi-asserted-by":"publisher","award":["RRF-2.3.1-21-2022-00003"],"award-info":[{"award-number":["RRF-2.3.1-21-2022-00003"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Fibrillin-1 microfibrils are essential elements of the extracellular matrix serving as a scaffold for the deposition of elastin and endowing connective tissues with tensile strength and elasticity. Mutations in the fibrillin-1 gene (FBN1) are linked to Marfan syndrome (MFS), a systemic connective tissue disorder that, besides other heterogeneous symptoms, usually manifests in life-threatening aortic complications. The aortic involvement may be explained by a dysregulation of microfibrillar function and, conceivably, alterations in the microfibrils\u2019 supramolecular structure. Here, we present a nanoscale structural characterization of fibrillin-1 microfibrils isolated from two human aortic samples with different FBN1 gene mutations by using atomic force microscopy, and their comparison with microfibrillar assemblies purified from four non-MFS human aortic samples. Fibrillin-1 microfibrils displayed a characteristic \u201cbeads-on-a-string\u201d appearance. The microfibrillar assemblies were investigated for bead geometry (height, length, and width), interbead region height, and periodicity. MFS fibrillin-1 microfibrils had a slightly higher mean bead height, but the bead length and width, as well as the interbead height, were significantly smaller in the MFS group. The mean periodicity varied around 50\u201352 nm among samples. The data suggest an overall thinner and presumably more frail structure for the MFS fibrillin-1 microfibrils, which may play a role in the development of MFS-related aortic symptomatology.<\/jats:p>","DOI":"10.3390\/ijms24087561","type":"journal-article","created":{"date-parts":[[2023,4,20]],"date-time":"2023-04-20T04:53:59Z","timestamp":1681966439000},"page":"7561","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta"],"prefix":"10.3390","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5622-9136","authenticated-orcid":false,"given":"Cristina M.","family":"\u0218ulea","sequence":"first","affiliation":[{"name":"Department of Biophysics and Radiation Biology, Semmelweis University, 1094 Budapest, Hungary"},{"name":"Heart and Vascular Center, Semmelweis University, 1122 Budapest, Hungary"},{"name":"Hungarian Marfan Foundation, 1122 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7820-8109","authenticated-orcid":false,"given":"Zsolt","family":"M\u00e1rtonfalvi","sequence":"additional","affiliation":[{"name":"Department of Biophysics and Radiation Biology, Semmelweis University, 1094 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Csilla","family":"Cs\u00e1nyi","sequence":"additional","affiliation":[{"name":"Department of Biophysics and Radiation Biology, Semmelweis University, 1094 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3760-2016","authenticated-orcid":false,"given":"D\u00f3ra","family":"Haluszka","sequence":"additional","affiliation":[{"name":"Department of Biophysics and Radiation Biology, Semmelweis University, 1094 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikl\u00f3s","family":"P\u00f3los","sequence":"additional","affiliation":[{"name":"Heart and Vascular Center, Semmelweis University, 1122 Budapest, Hungary"},{"name":"Hungarian Marfan Foundation, 1122 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6492-0426","authenticated-orcid":false,"given":"Bence","family":"\u00c1gg","sequence":"additional","affiliation":[{"name":"Heart and Vascular Center, Semmelweis University, 1122 Budapest, Hungary"},{"name":"Hungarian Marfan Foundation, 1122 Budapest, Hungary"},{"name":"Department of Pharmacology and Pharmacotherapy, Semmelweis University, 1089 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roland","family":"Stengl","sequence":"additional","affiliation":[{"name":"Heart and Vascular Center, Semmelweis University, 1122 Budapest, Hungary"},{"name":"Hungarian Marfan Foundation, 1122 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K\u00e1lm\u00e1n","family":"Benke","sequence":"additional","affiliation":[{"name":"Heart and Vascular Center, Semmelweis University, 1122 Budapest, Hungary"},{"name":"Hungarian Marfan Foundation, 1122 Budapest, Hungary"},{"name":"Department of Cardiac Surgery, University Hospital Halle (Saale), 06120 Halle (Saale), Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zolt\u00e1n","family":"Szabolcs","sequence":"additional","affiliation":[{"name":"Heart and Vascular Center, Semmelweis University, 1122 Budapest, Hungary"},{"name":"Hungarian Marfan Foundation, 1122 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikl\u00f3s S. Z.","family":"Kellermayer","sequence":"additional","affiliation":[{"name":"Department of Biophysics and Radiation Biology, Semmelweis University, 1094 Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,4,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1042\/BJ20101320","article-title":"TB domain proteins: Evolutionary insights into the multifaceted roles of fibrillins and LTBPs","volume":"433","author":"Robertson","year":"2011","journal-title":"Biochem. J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1038\/353395a0","article-title":"Calcium binding to fibrillin?","volume":"353","author":"Handford","year":"1991","journal-title":"Nature"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1006\/jmbi.1996.0003","article-title":"Calcium binding properties of an epidermal growth factor-like domain pair from human fibrillin-1","volume":"255","author":"Knott","year":"1996","journal-title":"J. Mol. Biol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1016\/S0092-8674(00)81259-3","article-title":"Solution structure of a pair of calcium-binding epidermal growth factor-like domains: Implications for the Marfan syndrome and other genetic disorders","volume":"85","author":"Downing","year":"1996","journal-title":"Cell"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"7368","DOI":"10.1074\/jbc.272.11.7368","article-title":"Calcium determines the shape of fibrillin","volume":"272","author":"Reinhardt","year":"1997","journal-title":"J. Biol. Chem."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1074\/jbc.272.2.1231","article-title":"Calcium stabilizes fibrillin-1 against proteolytic degradation","volume":"272","author":"Reinhardt","year":"1997","journal-title":"J. Biol. Chem."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"6743","DOI":"10.1074\/jbc.M607008200","article-title":"\u03b1v\u03b26 is a novel receptor for human fibrillin-1. Comparative studies of molecular determinants underlying integrin-RGD affinity and specificity","volume":"282","author":"Jovanovic","year":"2007","journal-title":"J. Biol. Chem."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/j.semcdb.2018.07.016","article-title":"Fibrillin microfibrils and elastic fibre proteins: Functional interactions and extracellular regulation of growth factors","volume":"89","author":"Thomson","year":"2019","journal-title":"Semin. Cell Dev. Biol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/s00441-009-0822-x","article-title":"Biogenesis and function of fibrillin assemblies","volume":"339","author":"Ramirez","year":"2010","journal-title":"Cell Tissue Res."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1111\/iep.12239","article-title":"Fell-Muir Lecture: Fibrillin microfibrils: Structural tensometers of elastic tissues?","volume":"98","author":"Kielty","year":"2017","journal-title":"Int. J. Exp. Pathol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1165","DOI":"10.1083\/jcb.129.4.1165","article-title":"Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils","volume":"129","author":"Zhang","year":"1995","journal-title":"J. Cell Biol."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Asano, K., Cantalupo, A., Sedes, L., and Ramirez, F. (2022). The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23031892"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"2499","DOI":"10.1083\/jcb.103.6.2499","article-title":"Fibrillin, a new 350-kD glycoprotein is a component of extracellular microfbrils","volume":"103","author":"Sakai","year":"1986","journal-title":"J. Cell Biol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.matbio.2019.06.006","article-title":"The role of fibrillin and microfibril binding proteins in elastin and elastic fibre assembly","volume":"84","author":"Godwin","year":"2019","journal-title":"Matrix Biol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1038\/icb.1987.39","article-title":"The immunohistochemical localisation of microfibril-associated glycoprotein (MAGP) in elastic and non-elastic tissues","volume":"65","author":"Gibson","year":"1987","journal-title":"Immunol. Cell Biol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/0014-5793(96)00423-1","article-title":"Fibrillin: Evidence that chondroitin sulphate proteoglycans are components of microfibrils and associate with newly synthesized monomers","volume":"386","author":"Kielty","year":"1996","journal-title":"FEBS Lett."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1002\/pmic.200401340","article-title":"Proteomic analysis of fibrillin-rich microfibrils","volume":"6","author":"Cain","year":"2006","journal-title":"Proteomics"},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Keeley, F., and Mecham, R. (2013). Evolution of Extracellular Matrix. Biology of Extracellular Matrix, Springer.","DOI":"10.1007\/978-3-642-36002-2"},{"key":"ref_19","first-page":"e002058","article-title":"Impact of Pathogenic FBN1 Variant Types on the Progression of Aortic Disease in Patients With Marfan Syndrome","volume":"11","author":"Takeda","year":"2018","journal-title":"Circ. Genom. Precis. Med."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/j.ijcard.2015.05.072","article-title":"Increased aortic tortuosity indicates a more severe aortic phenotype in adults with Marfan syndrome","volume":"194","author":"Franken","year":"2015","journal-title":"Int. J. Cardiol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1186\/s13023-020-01369-w","article-title":"Increased visceral arterial tortuosity in Marfan syndrome","volume":"15","author":"Szilveszter","year":"2020","journal-title":"Orphanet. J. Rare Dis."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"697591","DOI":"10.3389\/fcvm.2021.697591","article-title":"Case Report: Morphological Characterization and Long-Term Observation of Bilateral Sequential Internal Mammary Artery Aneurysms in a Patient With Confirmed FBN1 Mutation","volume":"8","author":"Stengl","year":"2021","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1136\/jmg.2009.072785","article-title":"The revised Ghent nosology for the Marfan syndrome","volume":"47","author":"Loeys","year":"2010","journal-title":"J. Med. Genet."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"633","DOI":"10.21037\/acs.2017.10.03","article-title":"Aortic dissection in patients with Marfan syndrome based on the IRAD data","volume":"6","author":"Trimarchi","year":"2017","journal-title":"Ann. Cardiothorac. Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1850","DOI":"10.1016\/j.athoracsur.2019.08.096","article-title":"Aortic Complications in Marfan Syndrome: Should We Anticipate Preventive Aortic Root Surgery?","volume":"109","author":"Evangelista","year":"2020","journal-title":"Ann. Thorac. Surg."},{"key":"ref_26","first-page":"696","article-title":"Changing strategies in aortic root reconstruction in Marfan syndrome","volume":"162","author":"Stengl","year":"2021","journal-title":"Orv. Hetil."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1186\/s13023-021-01882-6","article-title":"Potential predictors of severe cardiovascular involvement in Marfan syndrome: The emphasized role of genotype-phenotype correlations in improving risk stratification-a literature review","volume":"16","author":"Stengl","year":"2021","journal-title":"Orphanet. J. Rare Dis."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1007\/s00109-012-0931-y","article-title":"Exon 47 skipping of fibrillin-1 leads preferentially to cardiovascular defects in patients with thoracic aortic aneurysms and dissections","volume":"91","author":"Wang","year":"2013","journal-title":"J. Mol. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1111\/cge.12314","article-title":"Detection of 15 novel mutations in 52 children from 40 families with the Marfan or Loeys-Dietz syndrome and phenotype-genotype correlations","volume":"86","author":"Pees","year":"2014","journal-title":"Clin. Genet."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1038\/gim.2014.91","article-title":"Increased frequency of FBN1 truncating and splicing variants in Marfan syndrome patients with aortic events","volume":"17","author":"Baudhuin","year":"2015","journal-title":"Genet. Med."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"3285","DOI":"10.1093\/eurheartj\/ehv739","article-title":"Genotype impacts survival in Marfan syndrome","volume":"37","author":"Franken","year":"2016","journal-title":"Eur. Heart J."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"2739","DOI":"10.1093\/eurheartj\/ehx391","article-title":"2017 ESC\/EACTS Guidelines for the management of valvular heart disease","volume":"38","author":"Baumgartner","year":"2017","journal-title":"Eur. Heart J."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1186\/s13023-020-01569-4","article-title":"Optimising the mutation screening strategy in Marfan syndrome and identifying genotypes with more severe aortic involvement","volume":"15","author":"Stengl","year":"2020","journal-title":"Orphanet. J. Rare Dis."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"227","DOI":"10.5603\/CJ.2013.0066","article-title":"The role of transforming growth factor-beta in Marfan syndrome","volume":"20","author":"Benke","year":"2013","journal-title":"Cardiol. J."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/j.matbio.2017.07.004","article-title":"Marfan syndrome; A connective tissue disease at the crossroads of mechanotransduction, TGFbeta signaling and cell stemness","volume":"71\u201372","author":"Ramirez","year":"2018","journal-title":"Matrix Biol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1161\/CIRCGENETICS.114.000950","article-title":"Beneficial Outcome of Losartan Therapy Depends on Type of FBN1 Mutation in Marfan Syndrome","volume":"8","author":"Franken","year":"2015","journal-title":"Circ. Cardiovasc. Genet."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1517\/21678707.2014.950223","article-title":"Diagnosis and genetics of Marfan syndrome","volume":"2","author":"Franken","year":"2014","journal-title":"Expert. Opin. Orphan. Drugs"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"4565","DOI":"10.1074\/jbc.M110583200","article-title":"Versican interacts with fibrillin-1 and links extracellular microfibrils to other connective tissue networks","volume":"277","author":"Isogai","year":"2002","journal-title":"J. Biol. Chem."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"2750","DOI":"10.1074\/jbc.M209256200","article-title":"Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-associated protein","volume":"278","author":"Isogai","year":"2003","journal-title":"J. Biol. Chem."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1177\/44.12.8985131","article-title":"Microfibril-associated glycoprotein-1 (MAGP-1) is specifically located on the beads of the beaded-filament structure for fibrillin-containing microfibrils as visualized by the rotary shadowing technique","volume":"44","author":"Henderson","year":"1996","journal-title":"J. Histochem. Cytochem."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1016\/S0248-4900(98)80018-X","article-title":"Atomic force microscopy and modeling of natural elastic fibrillin polymers","volume":"90","author":"Hanssen","year":"1998","journal-title":"Biol. Cell"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1016\/S0065-3233(05)70012-7","article-title":"Fibrillin microfibrils","volume":"70","author":"Kielty","year":"2005","journal-title":"Adv. Protein. Chem."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"5117","DOI":"10.1074\/jbc.RA117.001483","article-title":"Structural and compositional diversity of fibrillin microfibrils in human tissues","volume":"293","author":"Eckersley","year":"2018","journal-title":"J. Biol. Chem."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1817","DOI":"10.1242\/jeb.199.8.1817","article-title":"Morphology and biomechanics of the microfibrillar network of sea cucumber dermis","volume":"199","author":"Thurmond","year":"1996","journal-title":"J. Exp. Biol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"4843","DOI":"10.1016\/j.actbio.2014.07.001","article-title":"Localized micro- and nano-scale remodelling in the diabetic aorta","volume":"10","author":"Akhtar","year":"2014","journal-title":"Acta Biomater."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1242\/jcs.108.3.1317","article-title":"Cysteine-to-arginine point mutation in a \u2018hybrid\u2019 eight-cysteine domain of FBN1: Consequences for fibrillin aggregation and microfibril assembly","volume":"108","author":"Kielty","year":"1995","journal-title":"J. Cell Sci."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/j.cellsig.2018.11.024","article-title":"Selective proteolysis by matrix metalloproteinases of photo-oxidised dermal extracellular matrix proteins","volume":"54","author":"Hibbert","year":"2019","journal-title":"Cell. Signal."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.rvsc.2020.09.006","article-title":"Investigation of fibrillin microfibrils in the canine cruciate ligament in dogs with different predispositions to ligament rupture","volume":"133","author":"Cox","year":"2020","journal-title":"Res. Vet. Sci."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1016\/S1357-2725(97)00028-9","article-title":"Scanning transmission electron microscopy mass analysis of fibrillin-containing microfibrils from foetal elastic tissues","volume":"29","author":"Sherratt","year":"1997","journal-title":"Int. J. Biochem. Cell. Biol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1016\/0889-1605(88)90039-0","article-title":"Vitreous humor of chicken contains two fibrillar systems: An analysis of their structure","volume":"100","author":"Wright","year":"1988","journal-title":"J. Ultrastruct. Mol. Struct. Res."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"99","DOI":"10.3109\/02713689109007614","article-title":"Rotary shadowing of elastic system microfibrils in the ocular zonule, vitreous, and ligamentum nuchae","volume":"10","author":"Wallace","year":"1991","journal-title":"Curr. Eye Res."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1083\/jcb.152.5.1045","article-title":"The supramolecular organization of fibrillin-rich microfibrils","volume":"152","author":"Baldock","year":"2001","journal-title":"J. Cell Biol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/S0022-2836(03)00829-5","article-title":"Fibrillin Microfibrils are Stiff Reinforcing Fibres in Compliant Tissues","volume":"332","author":"Sherratt","year":"2003","journal-title":"J. Mol. Biol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1161\/CIRCULATIONAHA.108.841981","article-title":"Circulating transforming growth factor-beta in Marfan syndrome","volume":"120","author":"Matt","year":"2009","journal-title":"Circulation"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1536\/ihj.54.23","article-title":"Circulating transforming growth factor beta-1 level in Japanese patients with Marfan syndrome","volume":"54","author":"Ogawa","year":"2013","journal-title":"Int. Heart J."},{"key":"ref_56","doi-asserted-by":"crossref","unstructured":"Agg, B., Benke, K., Szilveszter, B., Polos, M., Daroczi, L., Odler, B., Nagy, Z.B., Tarr, F., Merkely, B., and Szabolcs, Z. (2014). Possible extracardiac predictors of aortic dissection in Marfan syndrome. BMC Cardiovasc. Disord., 14.","DOI":"10.1186\/1471-2261-14-47"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1172\/JCI115589","article-title":"Marfan syndrome: Defective synthesis, secretion, and extracellular matrix formation of fibrillin by cultured dermal fibroblasts","volume":"89","author":"Milewicz","year":"1992","journal-title":"J. Clin. Investig."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"2269","DOI":"10.1093\/hmg\/ddg241","article-title":"Allelic variation in normal human FBN1 expression in a family with Marfan syndrome: A potential modifier of phenotype?","volume":"12","author":"Hutchinson","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1242\/jcs.99.4.797","article-title":"Isolation and ultrastructural analysis of microfibrillar structures from foetal bovine elastic tissues. Relative abundance and supramolecular architecture of type VI collagen assemblies and fibrillin","volume":"99","author":"Kielty","year":"1991","journal-title":"J. Cell Sci."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/24\/8\/7561\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T19:19:33Z","timestamp":1760123973000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/24\/8\/7561"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,20]]},"references-count":59,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2023,4]]}},"alternative-id":["ijms24087561"],"URL":"https:\/\/doi.org\/10.3390\/ijms24087561","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,20]]}}}