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<title>Fibrillin</title>

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<h1 id="firstHeading" class="firstHeading mw-first-heading"><span class="mw-page-title-main">Fibrillin</span></h1>

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<th colspan="3" style="background:#90EE90; color:#202122;">Fibrillin-1

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<td colspan="3" class="hintergrundfarbe1" style="text-align:center; font-size:smaller; font-weight:bold;">Bändermodell von Fibrillin-1, nach <a href="Protein_Data_Bank" title="Protein Data Bank">PDB</a> <a rel="nofollow" class="external text" href="https://www.rcsb.org/structure/2W86">2W86</a>

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<p>Vorhandene Strukturdaten: 2W86

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<th colspan="3" style="background:#90EE90; color:#202122;;">Eigenschaften des menschlichen Proteins

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<th colspan="3" style="background:#90EE90; color:#202122;">Bezeichner

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<td><a href="Human_Genome_Organisation" title="Human Genome Organisation">Gen-Namen</a>

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<td colspan="2" class="" style="text-align:center;"><i><a rel="nofollow" class="external text" href="https://www.genenames.org/tools/search/#!/all?query=3603">FBN1</a></i>, FBN, MFS1, WMS

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<td>Externe IDs

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<ul><li><a href="GeneCards" title="GeneCards">GeneCards</a>: <a rel="nofollow" class="external text" href="http://www.genecards.org/cgi-bin/carddisp.pl?gene=FBN1">FBN1</a></li>

<li><a href="UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P35555">P35555</a></li></ul>

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<th colspan="3" style="background:#90EE90; color:#202122;">Vorkommen

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<td style="background:#C3FDB8; color:#202122;">Homologie-Familie

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<td colspan="2" style="text-align:center;"><a rel="nofollow" class="external text" href="http://hogenom.univ-lyon1.fr/query_sequence?seq=P35555">Hovergen</a>

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</tbody></table><p><span class="editoronly" style="display:none;"></span>

</p><table class="wikitable hintergrundfarbe-basis infobox float-right" id="Vorlage_Infobox_Protein_Fibrillin-2" style="font-size:90%; margin-top:0; width:350px;" summary="Infobox Protein">

<th colspan="3" style="background:#90EE90; color:#202122;">Fibrillin-2

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<th colspan="3" style="background:#90EE90; color:#202122;;">Eigenschaften des menschlichen Proteins

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<th colspan="3" style="background:#90EE90; color:#202122;">Bezeichner

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<td><a href="Human_Genome_Organisation" title="Human Genome Organisation">Gen-Namen</a>

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<td colspan="2" class="" style="text-align:center;"><i><a rel="nofollow" class="external text" href="https://www.genenames.org/tools/search/#!/all?query=3604">FBN2</a></i>, CCA

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<td>Externe IDs

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<ul><li><a href="GeneCards" title="GeneCards">GeneCards</a>: <a rel="nofollow" class="external text" href="http://www.genecards.org/cgi-bin/carddisp.pl?gene=FBN2">FBN2</a></li>

<li><a href="UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P35556">P35556</a></li></ul>

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<th colspan="3" style="background:#90EE90; color:#202122;">Vorkommen

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<td style="background:#C3FDB8; color:#202122;">Homologie-Familie

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<td colspan="2" style="text-align:center;"><a rel="nofollow" class="external text" href="http://hogenom.univ-lyon1.fr/query_sequence?seq=P35556">Hovergen</a>

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</tbody></table><p><span class="editoronly" style="display:none;"></span>

</p><table class="wikitable hintergrundfarbe-basis infobox float-right" id="Vorlage_Infobox_Protein_Fibrillin-3" style="font-size:90%; margin-top:0; width:350px;" summary="Infobox Protein">

<th colspan="3" style="background:#90EE90; color:#202122;">Fibrillin-3

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<th colspan="3" style="background:#90EE90; color:#202122;;">Eigenschaften des menschlichen Proteins

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<th colspan="3" style="background:#90EE90; color:#202122;">Bezeichner

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<td>Externe IDs

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<ul><li><a href="GeneCards" title="GeneCards">GeneCards</a>: <a rel="nofollow" class="external text" href="http://www.genecards.org/cgi-bin/carddisp.pl?gene=FBN3">FBN3</a></li>

<li><a href="UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/Q75N90">Q75N90</a></li></ul>

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<th colspan="3" style="background:#90EE90; color:#202122;">Vorkommen

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<td style="background:#C3FDB8; color:#202122;">Homologie-Familie

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<td colspan="2" style="text-align:center;"><a rel="nofollow" class="external text" href="http://hogenom.univ-lyon1.fr/query_sequence?seq=Q75N90">Hovergen</a>

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</p><p><b>Fibrillin</b> ist ein für die Bildung <a href="Elastische_Faser" title="Elastische Faser">elastischer Fasern</a> essenzielles <a href="Glykoproteine" title="Glykoproteine">Glykoprotein</a>, das im <a href="Bindegewebe" title="Bindegewebe">Bindegewebe</a> vorkommt.<sup id="cite_ref-1" class="reference"><a href="#cite_note-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> Fibrillin wird von <a href="Fibroblast" title="Fibroblast">Fibroblasten</a> in die <a href="Extrazellul%C3%A4re_Matrix" title="Extrazelluläre Matrix">extrazelluläre Matrix</a> <a href="Sekretion" title="Sekretion">sezerniert</a>. Aus ihnen entstehen <a href="Mikrofibrille" title="Mikrofibrille">Mikrofibrillen</a>, die als Gerüststruktur für die Einlagerung von <a href="Elastin" title="Elastin">Elastin</a> dienen.<sup id="cite_ref-2" class="reference"><a href="#cite_note-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup>

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<div class="mw-heading mw-heading2"><h2 id="Eigenschaften">Eigenschaften</h2></div>

<p>Die Kontrolle der <a href="Bioverf%C3%BCgbarkeit" title="Bioverfügbarkeit">Bioverfügbarkeit</a> des Wachstumsfaktors <a href="TGF-%CE%B2" class="mw-redirect" title="TGF-β">TGF-β</a> ist wichtig für die Gewebebildung und der <a href="Hom%C3%B6ostase" title="Homöostase">Homöostase</a>. Die Ablagerung des latenten TGF-β-Komplexes (LLC) in der extrazellulären Matrix wird durch Fibrillin-Mikrofibrillen verursacht. Dabei interagiert das in dem Komplex befindliche Protein LTBP-1 <i>(Latent TGFβ-binding protein 1)</i> mit <a href="Fibronektin" title="Fibronektin">Fibronektin</a> während der Fibrillogenese.<sup id="cite_ref-3" class="reference"><a href="#cite_note-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> Außerdem beteiligen sich Fibrilline an der strukturellen Erhaltung der extrazellulären Matrix, indem sie sich an BMP7-ähnlichen Wachstumsfaktoren binden.<sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup>

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<div class="mw-heading mw-heading2"><h2 id="Klinische_Bedeutung">Klinische Bedeutung</h2></div>

<p>Mutationen in FBN1 und FBN2 stehen im Zusammenhang mit <a href="Skoliose" title="Skoliose">Skoliose</a>.<sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup>

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<div class="mw-heading mw-heading2"><h2 id="Arten">Arten</h2></div>

<div class="mw-heading mw-heading3"><h3 id="Fibrillin-1">Fibrillin-1</h3></div>

<p>Fibrillin-1 ist der Hauptbestandteil der Mikrofibrillen, die im Falle der <a href="Elastische_Faser" title="Elastische Faser">elastischen Fasern</a> wie ein Mantel den amorphen Kern aus Elastin umgeben. Bisher wurden vier Formen von Fibrillinen beschrieben. Fibrillin-1 wurde durch Engvall 1986 isoliert,<sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> und Mutationen im FBN1-Gen sind für das <a href="Marfan-Syndrom" title="Marfan-Syndrom">Marfan-Syndrom</a> verantwortlich.<sup id="cite_ref-7" class="reference"><a href="#cite_note-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-8" class="reference"><a href="#cite_note-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup>

</p><p>Das Protein kommt im Menschen vor und sein Gen befindet sich auf <a href="Chromosom_15_(Mensch)" title="Chromosom 15 (Mensch)">Chromosom 15</a>. Bisher wurden mehr als 1500 verschiedene Mutationen beschrieben.

</p><p><b>Struktur</b><br>

Bisher gibt es keine vollständige, hochaufgelöste Struktur von Fibrillin-1. Es wurden jedoch kürzere Fragmente rekombinant hergestellt und ihre Strukturen mit <a href="R%C3%B6ntgenkristallographie" class="mw-redirect" title="Röntgenkristallographie">Röntgenkristallographie</a> bzw. <a href="Kernspinresonanzspektroskopie" title="Kernspinresonanzspektroskopie">NMR-Spektroskopie</a> aufgeklärt. Ein neueres Beispiel ist die Struktur der Fibrillin-1-Hybrid-Domäne in Bezug auf die kalziumbindende epidermale Wachstumsfaktordomäne. Diese wurde mittels Röntgenkristallographie und einer Auflösung von 1,8 Å bestimmt. Die Mikrofibillen, die aus Fibrillinproteinen bestehen, sind bedeutsam für unterschiedliche Interaktionen zwischen Zellen und der <a href="Extrazellul%C3%A4re_Matrix" title="Extrazelluläre Matrix">extrazellulären Matrix</a>.

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<div class="mw-heading mw-heading3"><h3 id="Fibrillin-2">Fibrillin-2</h3></div>

<p>Fibrillin-2 wurde erstmals 1994 von Zhang<sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> isoliert. Es wird vermutet, dass es eine wesentliche Rolle in der frühen Phase der Elastogenese (Bildung elastischer Fasern) spielt. Mutationen im Fibrillin-2-Gen wurden mit dem <a href="Kongenitale_Kontrakturale_Arachnodaktylie" title="Kongenitale Kontrakturale Arachnodaktylie">Beals-Hecht-Syndrom</a> in Verbindung gebracht.

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<div class="mw-heading mw-heading3"><h3 id="Fibrillin-3">Fibrillin-3</h3></div>

<p>Für Fibrillin-3 wird angenommen, dass es hauptsächlich im Gehirn vorkommt.<sup id="cite_ref-10" class="reference"><a href="#cite_note-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> Es wurde außerdem für die Feldmaus gezeigt, dass Fibrillin-3 in den Eierstöcken und in der Keimdrüse vorkommt.

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<div class="mw-heading mw-heading3"><h3 id="Fibrillin-4">Fibrillin-4</h3></div>

<p>Fibrillin-4 wurde erstmals im <a href="Zebrab%C3%A4rbling" title="Zebrabärbling">Zebrabärbling</a> entdeckt, seine Sequenz ähnelt sehr der des Fibillin-2.<sup id="cite_ref-11" class="reference"><a href="#cite_note-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup>

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<div class="mw-heading mw-heading2"><h2 id="Einzelnachweise">Einzelnachweise</h2></div>

<li id="cite_note-1"><span class="mw-cite-backlink"><a href="#cite_ref-1">↑</a></span> <span class="reference-text">C. M. Kielty, C. Baldock, D. Lee, M. J. Rock, J. L. Ashworth, C. A. Shuttleworth: <cite style="font-style:italic">Fibrillin: from microfibril assembly to biomechanical function.</cite> In: <cite style="font-style:italic">Philosophical Transactions of the Royal Society B: Biological Sciences</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>357</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>1418</span>, 2002, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%220962-8436%22&key=cql">0962-8436</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>207–217</span>, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.1098/rstb.2001.1029">10.1098/rstb.2001.1029</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/11911778?dopt=Abstract">PMID 11911778</a>, <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1692929/">PMC 1692929</a> (freier Volltext).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=Fibrillin%3A+from+microfibril+assembly+to+biomechanical+function.&rft.au=C.+M.+Kielty%2C+C.+Baldock%2C+D.+Lee%2C+...&rft.date=2002&rft.doi=10.1098%2Frstb.2001.1029&rft.genre=journal&rft.issn=0962-8436&rft.issue=1418&rft.jtitle=Philosophical+Transactions+of+the+Royal+Society+B%3A+Biological+Sciences&rft.pages=207-217&rft.pmc=1692929&rft.pmid=11911778&rft.volume=357" style="display:none"> </span></span>

</li>

<li id="cite_note-2"><span class="mw-cite-backlink"><a href="#cite_ref-2">↑</a></span> <span class="reference-text">Inderbir Singh: <cite style="font-style:italic">Textbook of human histology</cite>. 5. Auflage. Jaypee Brothers, Neu-Delhi 2006, ISBN 81-8061-809-9, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>64<span style="display:inline-block;width:.2em"> </span>ff</span>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.au=Inderbir+Singh&rft.btitle=Textbook+of+human+histology&rft.date=2006&rft.edition=5&rft.genre=book&rft.isbn=8180618099&rft.pages=64ff&rft.place=Neu-Delhi&rft.pub=Jaypee+Brothers" style="display:none"> </span></span>

</li>

<li id="cite_note-3"><span class="mw-cite-backlink"><a href="#cite_ref-3">↑</a></span> <span class="reference-text">T. Massam-Wu, M. Chiu, R. Choudhury, S. S. Chaudhry, A. K. Baldwin, A. McGovern, C. Baldock, C. A. Shuttleworth, C. M. Kielty: <cite style="font-style:italic">Assembly of fibrillin microfibrils governs extracellular deposition of latent TGFβ</cite>. In: <cite style="font-style:italic">Journal of Cell Science</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>123</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>17</span>, 2010, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%220021-9533%22&key=cql">0021-9533</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>3006–3018</span>, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.1242/jcs.073437">10.1242/jcs.073437</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/20699357?dopt=Abstract">PMID 20699357</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=Assembly+of+fibrillin+microfibrils+governs+extracellular+deposition+of+latent+TGF%CE%B2&rft.au=T.+Massam-Wu%2C+M.+Chiu%2C+R.+Choudhury%2C+...&rft.date=2010&rft.doi=10.1242%2Fjcs.073437&rft.genre=journal&rft.issn=0021-9533&rft.issue=17&rft.jtitle=Journal+of+Cell+Science&rft.pages=3006-3018&rft.pmid=20699357&rft.volume=123" style="display:none"> </span></span>

</li>

<li id="cite_note-4"><span class="mw-cite-backlink"><a href="#cite_ref-4">↑</a></span> <span class="reference-text">Ian Robertson, Sacha Jensen, Penny Handford: <cite style="font-style:italic">TB domain proteins: evolutionary insights into the multifaceted roles of fibrillins and LTBPs</cite>. In: <cite style="font-style:italic">Biochemical Journal</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>433</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>2</span>, 2011, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%220264-6021%22&key=cql">0264-6021</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>263–276</span>, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.1042/BJ20101320">10.1042/BJ20101320</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/21175431?dopt=Abstract">PMID 21175431</a> (<a rel="nofollow" class="external text" href="http://www.biochemj.org/content/433/2/263">biochemj.org</a>).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=TB+domain+proteins%3A+evolutionary+insights+into+the+multifaceted+roles+of+fibrillins+and+LTBPs&rft.au=Ian+Robertson%2C+Sacha+Jensen%2C+Penny+Handford&rft.date=2011&rft.doi=10.1042%2FBJ20101320&rft.genre=journal&rft.issn=0264-6021&rft.issue=2&rft.jtitle=Biochemical+Journal&rft.pages=263-276&rft.pmid=21175431&rft.volume=433" style="display:none"> </span></span>

</li>

<li id="cite_note-5"><span class="mw-cite-backlink"><a href="#cite_ref-5">↑</a></span> <span class="reference-text">J. G. Buchan, D. M. Alvarado, G. E. Haller, C Cruchaga, M. B. Harms, T Zhang, M. C. Willing, D. K. Grange, A. C. Braverman, N. H. Miller, J. A. Morcuende, N. L. Tang, T. P. Lam, B. K. Ng, J. C. Cheng, M. B. Dobbs, C. A. Gurnett: <cite style="font-style:italic">Rare variants in FBN1 and FBN2 are associated with severe adolescent idiopathic scoliosis</cite>. In: <cite style="font-style:italic">Human Molecular Genetics</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>23</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>19</span>, 2014, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%221460-2083%22&key=cql">1460-2083</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>5271–5282</span>, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.1093/hmg%2Fddu224">10.1093/hmg/ddu224</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/24833718?dopt=Abstract">PMID 24833718</a>, <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4159151/">PMC 4159151</a> (freier Volltext).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=Rare+variants+in+FBN1+and+FBN2+are+associated+with+severe+adolescent+idiopathic+scoliosis&rft.au=J.+G.+Buchan%2C+D.+M.+Alvarado%2C+G.+E.+Haller%2C+...&rft.date=2014&rft.doi=10.1093%2Fhmg%2Fddu224&rft.genre=journal&rft.issn=1460-2083&rft.issue=19&rft.jtitle=Human+Molecular+Genetics&rft.pages=5271-5282&rft.pmc=4159151&rft.pmid=24833718&rft.volume=23" style="display:none"> </span></span>

</li>

<li id="cite_note-6"><span class="mw-cite-backlink"><a href="#cite_ref-6">↑</a></span> <span class="reference-text">L. Y. Sakai: <cite style="font-style:italic">Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils</cite>. In: <cite style="font-style:italic">The Journal of Cell Biology</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>103</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>6</span>, Dezember 1986, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%220021-9525%22&key=cql">0021-9525</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>2499–2509</span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/3536967?dopt=Abstract">PMID 3536967</a>, <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2114568/">PMC 2114568</a> (freier Volltext).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=Fibrillin%2C+a+new+350-kD+glycoprotein%2C+is+a+component+of+extracellular+microfibrils&rft.au=L.+Y.+Sakai&rft.date=1986-12&rft.genre=journal&rft.issn=0021-9525&rft.issue=6&rft.jtitle=The+Journal+of+Cell+Biology&rft.pages=2499-2509&rft.pmc=2114568&rft.pmid=3536967&rft.volume=103" style="display:none"> </span></span>

</li>

<li id="cite_note-7"><span class="mw-cite-backlink"><a href="#cite_ref-7">↑</a></span> <span class="reference-text">Alan E. Guttmacher, Harry C. Dietz: <cite style="font-style:italic">New therapeutic approaches to mendelian disorders</cite>. In: <cite style="font-style:italic">The New England Journal of Medicine</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>363</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>9</span>, August 2010, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%221533-4406%22&key=cql">1533-4406</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>852–863</span>, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.1056/NEJMra0907180">10.1056/NEJMra0907180</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/20818846?dopt=Abstract">PMID 20818846</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=New+therapeutic+approaches+to+mendelian+disorders&rft.au=Alan+E.+Guttmacher%2C+Harry+C.+Dietz&rft.date=2010-08&rft.doi=10.1056%2FNEJMra0907180&rft.genre=journal&rft.issn=1533-4406&rft.issue=9&rft.jtitle=The+New+England+Journal+of+Medicine&rft.pages=852-863&rft.pmid=20818846&rft.volume=363" style="display:none"> </span></span>

</li>

<li id="cite_note-8"><span class="mw-cite-backlink"><a href="#cite_ref-8">↑</a></span> <span class="reference-text">Y. von Kodolitsch: <cite style="font-style:italic">Perspectives on the revised Ghent criteria for the diagnosis of Marfan syndrome</cite>. In: <cite style="font-style:italic">The Application of Clinical Genetics</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>8</span>, 2015, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%221178-704X%22&key=cql">1178-704X</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>137–155</span>, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.2147/TACG.S60472">10.2147/TACG.S60472</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/26124674?dopt=Abstract">PMID 26124674</a>, <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4476478/">PMC 4476478</a> (freier Volltext).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=Perspectives+on+the+revised+Ghent+criteria+for+the+diagnosis+of+Marfan+syndrome&rft.au=Y.+von+Kodolitsch&rft.date=2015&rft.doi=10.2147%2FTACG.S60472&rft.genre=journal&rft.issn=1178-704X&rft.jtitle=The+Application+of+Clinical+Genetics&rft.pages=137-155&rft.pmc=4476478&rft.pmid=26124674&rft.volume=8" style="display:none"> </span></span>

</li>

<li id="cite_note-9"><span class="mw-cite-backlink"><a href="#cite_ref-9">↑</a></span> <span class="reference-text">H. Zhang, S. D. Apfelroth, W. Hu; E. C. Davis, C. Sanguineti, J. Bonadio, R. P. Mecham, F. Ramirez: <cite style="font-style:italic">Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices</cite>. In: <cite style="font-style:italic">The Journal of Cell Biology</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>124</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>5</span>, 1994, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%220021-9525%22&key=cql">0021-9525</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>855–863</span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/8120105?dopt=Abstract">PMID 8120105</a>, <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2119952/">PMC 2119952</a> (freier Volltext).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=Structure+and+expression+of+fibrillin-2%2C+a+novel+microfibrillar+component+preferentially+located+in+elastic+matrices&rft.au=H.+Zhang%2C+S.+D.+Apfelroth%2C+W.+Hu%3B+E.+C.+Davis%2C+...&rft.date=1994&rft.genre=journal&rft.issn=0021-9525&rft.issue=5&rft.jtitle=The+Journal+of+Cell+Biology&rft.pages=855-863&rft.pmc=2119952&rft.pmid=8120105&rft.volume=124" style="display:none"> </span></span>

</li>

<li id="cite_note-10"><span class="mw-cite-backlink"><a href="#cite_ref-10">↑</a></span> <span class="reference-text">G. M. Corson, N. L. Charbonneau, D. R. Keene, L. Y. Sakai: <cite style="font-style:italic">Differential expression of fibrillin-3 adds to microfibril variety in human and avian, but not rodent, connective tissues</cite>. In: <cite style="font-style:italic">Genomics</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>83</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>3</span>, März 2004, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%220888-7543%22&key=cql">0888-7543</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>461–472</span>, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.1016/j.ygeno.2003.08.023">10.1016/j.ygeno.2003.08.023</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/14962672?dopt=Abstract">PMID 14962672</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=Differential+expression+of+fibrillin-3+adds+to+microfibril+variety+in+human+and+avian%2C+but+not+rodent%2C+connective+tissues&rft.au=G.+M.+Corson%2C+N.+L.+Charbonneau%2C+D.+R.+Keene%2C+...&rft.date=2004-03&rft.doi=10.1016%2Fj.ygeno.2003.08.023&rft.genre=journal&rft.issn=0888-7543&rft.issue=3&rft.jtitle=Genomics&rft.pages=461-472&rft.pmid=14962672&rft.volume=83" style="display:none"> </span></span>

</li>

<li id="cite_note-11"><span class="mw-cite-backlink"><a href="#cite_ref-11">↑</a></span> <span class="reference-text">John M. Gansner, Erik C. Madsen, Robert P. Mecham, Jonathan D. Gitlin: <cite style="font-style:italic">Essential role for fibrillin-2 in zebrafish notochord and vascular morphogenesis</cite>. In: <cite style="font-style:italic">Developmental Dynamics. An Official Publication of the American Association of Anatomists</cite>. <span style="white-space:nowrap">Band<span style="display:inline-block;width:.2em"> </span>237</span>, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>10</span>, Oktober 2008, <a href="Internationale_Standardnummer_f%C3%BCr_fortlaufende_Sammelwerke" title="Internationale Standardnummer für fortlaufende Sammelwerke">ISSN</a> <span style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://zdb-katalog.de/list.xhtml?t=iss%3D%221058-8388%22&key=cql">1058-8388</a></span>, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>2844–2861</span>, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.1002/dvdy.21705">10.1002/dvdy.21705</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/18816837?dopt=Abstract">PMID 18816837</a>, <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3081706/">PMC 3081706</a> (freier Volltext).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Fibrillin&rft.atitle=Essential+role+for+fibrillin-2+in+zebrafish+notochord+and+vascular+morphogenesis&rft.au=John+M.+Gansner%2C+Erik+C.+Madsen%2C+Robert+P.+Mecham%2C+...&rft.date=2008-10&rft.doi=10.1002%2Fdvdy.21705&rft.genre=journal&rft.issn=1058-8388&rft.issue=10&rft.jtitle=Developmental+Dynamics.+An+Official+Publication+of+the+American+Association+of+Anatomists&rft.pages=2844-2861&rft.pmc=3081706&rft.pmid=18816837&rft.volume=237" style="display:none"> </span></span>

</li>

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