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Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblasts - PubMed

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Fibulin-4 is an elastic-fibre-associated glycoprotein that is indispensable for elastic-fibre formation in mice. However, the mechanism by which fibulin-4 executes this function remains to be …"><meta name="keywords" content="pmid:19627254, PMC3024593, doi:10.1042/BJ20090993, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov&#x27;t, Qiuyun Chen, Teng Zhang, Paul E Dicorleto, Amino Acid Sequence, Animals, CHO Cells, Cells, Cultured, Child, Preschool, Cricetinae, Cricetulus, Elastic Tissue / metabolism*, Elastic Tissue / physiology, Embryo, Mammalian, Extracellular Matrix Proteins / genetics, Extracellular Matrix Proteins / metabolism, Extracellular Matrix Proteins / physiology*, Fibroblasts / metabolism, Fibroblasts / physiology*, Gene Expression Regulation, Humans, Male, Mice, Mice, Knockout, Molecular Sequence Data, Tropoelastin / genetics*, Tropoelastin / metabolism, PubMed Abstract, NIH, NLM, NCBI, National Institutes of Health, National Center for Biotechnology Information, National Library of Medicine, MEDLINE"><meta name="robots" content="index,nofollow,noarchive"><meta property="og:title" content="Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblasts - PubMed"><meta property="og:url" content="https://pubmed.ncbi.nlm.nih.gov/19627254/"><meta property="og:description" content="Elastic fibres are essential for normal physiology in numerous tissues, including arteries, lungs and skin. 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However, the mechanism by which fibulin-4 executes this function remains to be …"><meta name="twitter:image" content="https://cdn.ncbi.nlm.nih.gov/pubmed/persistent/pubmed-meta-image-v2.jpg"> <!-- OpenSearch XML --> <link rel="search" type="application/opensearchdescription+xml" href="https://cdn.ncbi.nlm.nih.gov/pubmed/persistent/opensearch.xml" title="PubMed search"> <!-- Disables severely broken elements when no JS --> <noscript> <link rel="stylesheet" type="text/css" href="https://cdn.ncbi.nlm.nih.gov/pubmed/14efe001-aebf-4097-9883-28c967333423/core/no-script.css"> </noscript> <meta name="citation_title" content="Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblasts"> <meta name="citation_authors" content="Chen Q;Zhang T;Roshetsky JF;Ouyang Z;Essers J;Fan C;Wang Q;Hinek A;Plow EF;Dicorleto PE;"> <meta name="citation_date" content="09/14/2009"> <meta name="citation_publisher" content="Biochem J"> <meta name="citation_journal_title" content="The Biochemical journal"> <meta name="citation_pmid" content="19627254"> <meta name="citation_abstract_html_url" content="https://pubmed.ncbi.nlm.nih.gov/19627254/"> <meta name="citation_volume" content="423"> <meta name="citation_issue" content="1"> <meta name="citation_doi" content="10.1042/BJ20090993"> <meta name="citation_issn" content="1470-8728"> <link rel="canonical" href="https://pubmed.ncbi.nlm.nih.gov/19627254/" /> </head> <body > <noscript> <div class="no-script-banner" id="no-script-banner"> <div class="warning-message"> <div class="warning-message-text"> This site needs JavaScript to work properly. 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class="collections-new" name="collections" value="new" data-ga-category="save_share" data-ga-action="collections" data-ga-label="collections_radio_new"> <label for="collections-action-panel-new">Create a new collection</label> </li> <li> <input type="radio" id="collections-action-panel-existing" class="collections-existing" name="collections" value="existing" checked="true" data-ga-category="save_share" data-ga-action="collections" data-ga-label="collections_radio_existing"> <label for="collections-action-panel-existing">Add to an existing collection</label> </li> </ul> </div> <div class="controls-wrapper"> <div class="action-panel-control-wrap new-collections-controls"> <label for="collections-action-panel-add-to-new" class="action-panel-label required-field-asterisk"> Name your collection: </label> <input type="text" name="add-to-new-collection" id="collections-action-panel-add-to-new" class="collections-action-add-to-new" pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/" maxlength="100" data-ga-category="save_share" data-ga-action="create_collection" data-ga-label="non_favorties_collection"> <div class="collections-new-name-too-long usa-input-error-message selection-validation-message"> Name must be less than 100 characters </div> </div> <div class="action-panel-control-wrap existing-collections-controls"> <label for="collections-action-panel-add-to-existing" class="action-panel-label"> Choose a collection: </label> <select id="collections-action-panel-add-to-existing" class="action-panel-selector collections-action-add-to-existing" data-ga-category="save_share" data-ga-action="select_collection" data-ga-label="($('#collections-action-add-to-existing').val() === 'Favorites') ? 'Favorites' : 'non_favorites_collection'"> </select> <div class="collections-retry-load-on-error usa-input-error-message selection-validation-message"> Unable to load your collection due to an error<br> <a href="#">Please try again</a> </div> </div> </div> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Adding..." data-pinger-ignore data-ga-category="save_share" data-ga-action="collections" data-ga-label="add"> Add </button> <button class="action-panel-cancel" aria-label="Close 'Add to Collections' panel" ref="linksrc=close_collections_panel" aria-controls="collections-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="collections" data-ga-label="cancel"> Cancel </button> </div> </form> </div> </div> <div id="bibliography-action-panel" class="bibliography-action-panel action-panel in-progress-dots-panel" aria-hidden="true" data-bibliography-open-panel-enabled="false" data-bibliography-open-panel-url-hash="#open-bibliography-panel"> <div class="inner-wrap"> <h3 class="action-panel-heading"> Add to My Bibliography </h3> <form id="bibliography-action-panel-form" class="bibliography-action-panel-form action-panel-content action-form action-panel-smaller-selectors" data-add-to-bibliography-max-amount="100" data-add-to-bibliography-batch-size="10" data-bibliography-delegates-url="/list-bibliography-delegates/" data-add-to-bibliography-url="/add-to-bibliography/" data-get-article-ids-by-search-url="/get-article-ids-by-search/" data-mybib-root-url="https://www.ncbi.nlm.nih.gov/myncbi/collections/mybibliography/"> <input type="hidden" name="csrfmiddlewaretoken" value="atdnHNu4lrgiFwPfwLPmrLj74UWCiBQAmnmCe5ey5mt5C60mn5ghRFYZeplqNTQG"> <div class="action-panel-control-wrap bibliographies-controls"> <div class="choice-group"> <ul class="bibliographies-action-add radio-group-items"> <li> <input name="bibliography" id="my-bibliography" class="my-bibliography" type="radio" checked/> <label for="my-bibliography">My Bibliography</label> </li> </ul> </div> </div> <div class="bibliographies-retry-load-on-error usa-input-error-message selection-validation-message"> Unable to load your delegates due to an error<br> <a href="#">Please try again</a> </div> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Adding..." data-pinger-ignore> Add </button> <button class="action-panel-cancel" aria-label="Close 'Add to bibliography' panel" ref="linksrc=close_bibliography_panel" aria-controls="bibliography-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="mybib" data-ga-label="cancel"> Cancel </button> </div> </form> </div> </div> <div id="saved-search-action-panel" class="saved-search-action-panel action-panel " aria-hidden="true" data-saved-search-open-panel-enabled="false" data-saved-search-open-panel-url-hash="#open-saved-search-panel"> <div class="inner-wrap"> <h2 class="action-panel-heading"> Your saved search </h2> <form id="saved-search-action-panel-form" class="saved-search-action-panel-form action-panel-content action-form" data-create-saved-search-url="/create-saved-search/" data-try-search-terms-url="/try-search-term/" data-saved-search-root-url="https://www.ncbi.nlm.nih.gov/myncbi/searches/"> <input type="hidden" name="csrfmiddlewaretoken" value="atdnHNu4lrgiFwPfwLPmrLj74UWCiBQAmnmCe5ey5mt5C60mn5ghRFYZeplqNTQG"> <div class="action-panel-control-wrap"> <label for="saved-search-name" class="action-panel-label saved-search-name-label required-field-asterisk"> Name of saved search: </label> <input maxlength="200" type="text" name="saved-search-name" id="saved-search-name" class="saved-search-name" value="" required pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/"> </div> <div class="action-panel-control-wrap"> <label for="saved-search-term" class="action-panel-label required-field-asterisk"> Search terms: </label> <textarea name="saved-search-term" id="saved-search-term" class="saved-search-term" required></textarea> </div> <div class="test-search-term-wrap"> <a href="#" class="try-search-term">Test search terms</a> </div> <div class="choice-group action-panel-extra-margin-top"> <span class="action-panel-label" id="fieldset-label"> Would you like email updates of new search results? </span> <fieldset id="saved-search-alert" aria-describedby="fieldset-label"> <legend class="usa-sr-only">Saved Search Alert Radio Buttons</legend> <ul class="radio-group-items"> <li> <input type="radio" id="saved-search-alert-yes" class="saved-search-alert-yes" name="saved-search-alert" value="yes" checked> <label for="saved-search-alert-yes" class="action-panel-label">Yes</label> </li> <li> <input aria-label="No radio input" type="radio" id="saved-search-alert-no" class="saved-search-alert-no" name="saved-search-alert" value="no"> <label for="saved-search-alert-no" class="action-panel-label">No</label> </li> </ul> </fieldset> </div> <div class="alert-schedule-wrap"> <div class="action-panel-control-wrap"> <label class="action-panel-label"> Email: </label> <span aria-label="Email address" id="saved-search-email" class="action-panel-label"><span class="action-panel-label-bold"></span> (<a class="myncbi-account-settings" href="https://www.ncbi.nlm.nih.gov/account/settings/">change</a>)</span> </div> <div class="action-panel-control-wrap action-panel-extra-margin-top"> <label for="saved-search-frequency" class="action-panel-label"> Frequency: </label> <select id="saved-search-frequency" class="no-border-panel-selector saved-search-frequency"> <option value="monthly">Monthly</option> <option value="weekly">Weekly</option> <option value="daily">Daily</option> </select> </div> <div class="action-panel-control-wrap saved-search-monthly-additional"> <label for="saved-search-monthly-on-day" class="action-panel-label"> Which day? </label> <select id="saved-search-monthly-on-day" class="no-border-panel-selector"> <option value="Sunday">The first Sunday</option> <option value="Monday">The first Monday</option> <option value="Tuesday">The first Tuesday</option> <option value="Wednesday">The first Wednesday</option> <option value="Thursday">The first Thursday</option> <option value="Friday">The first Friday</option> <option value="Saturday">The first Saturday</option> <option value="day">The first day</option> <option value="weekday">The first weekday</option> </select> </div> <div class="action-panel-control-wrap saved-search-weekly-additional"> <label for="saved-search-weekly-on-day" class="action-panel-label"> Which day? </label> <select id="saved-search-weekly-on-day" class="no-border-panel-selector saved-search-weekly-on-day"> <option value="Sunday">Sunday</option> <option value="Monday">Monday</option> <option value="Tuesday">Tuesday</option> <option value="Wednesday">Wednesday</option> <option value="Thursday">Thursday</option> <option value="Friday">Friday</option> <option value="Saturday">Saturday</option> </select> </div> <div class="action-panel-control-wrap"> <label for="saved-search-report" class="action-panel-label"> Report format: </label> <select id="saved-search-report" class="no-border-panel-selector saved-search-report"> <option value="DocSum">Summary</option> <option value="DocSumText">Summary (text)</option> <option value="Abstract">Abstract</option> <option value="AbstractText">Abstract (text)</option> <option value="MEDLINE">PubMed</option> </select> </div> <div class="action-panel-control-wrap"> <label for="saved-search-amount" class="action-panel-label"> Send at most: </label> <select id="saved-search-amount" class="no-border-panel-selector saved-search-amount"> <option value="1">1 item</option> <option value="5" selected>5 items</option> <option value="10">10 items</option> <option value="20">20 items</option> <option value="50">50 items</option> <option value="100">100 items</option> <option value="200">200 items</option> </select> </div> <div> <input type="checkbox" id="saved-search-send-if-no-result" class="saved-search-send-if-no-result" name="saved-search-send-if-no-result"> <label for="saved-search-send-if-no-result" class="action-panel-label smaller-checkbox"> Send even when there aren't any new results </label> </div> <div class="action-panel-control-wrap option-text-in-email-wrap"> <label for="saved-search-email-text" class="action-panel-label"> Optional text in email: </label> <textarea name="saved-search-email-text" id="saved-search-email-text" class="saved-search-email-text"></textarea> </div> </div> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Saving..." data-ga-category="save_share" data-ga-action="alert" data-ga-label="save"> Save </button> <button class="action-panel-cancel" aria-label="Close 'Your saved search' panel" ref="linksrc=close_saved_search_panel" aria-controls="saved-search-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="alert" data-ga-label="cancel"> Cancel </button> </div> </form> </div> </div> <div id="citation-manager-action-panel" class="citation-manager-action-panel action-panel" aria-hidden="true"> <div class="inner-wrap"> <h2 class="action-panel-heading"> Create a file for external citation management software </h2> <form id="citation-manager-action-panel-form" class="action-panel-content action-form" action="/results-export-ids/" data-by-search-action="/results-export-search-data/" data-by-ids-action="/results-export-ids/" method="post" data-by-search-method="post" data-by-ids-method="post"> <input type="hidden" name="csrfmiddlewaretoken" value="atdnHNu4lrgiFwPfwLPmrLj74UWCiBQAmnmCe5ey5mt5C60mn5ghRFYZeplqNTQG"> <input name="results-format" type="hidden" value="pubmed"/> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Sending..." data-ga-category="save_share" data-ga-action="citation_manager" data-ga-label="save"> Create file </button> <button class="action-panel-cancel" aria-label="Close 'Send citations to citation manager' panel" ref="linksrc=close_citation_manager_panel" aria-controls="citation-manager-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="citation_manager" data-ga-label="cancel"> Cancel </button> </div> </form> </div> </div> <div id="rss-action-panel" class="rss-action-panel action-panel " aria-hidden="true"> <div class="inner-wrap"> <h2 class="action-panel-heading"> Your RSS Feed </h2> <form id="rss-action-panel-form" class="rss-action-panel-form action-panel-content action-form" data-create-rss-feed-url="/create-rss-feed-url/" data-search-form-term-value=""> <input type="hidden" name="csrfmiddlewaretoken" value="atdnHNu4lrgiFwPfwLPmrLj74UWCiBQAmnmCe5ey5mt5C60mn5ghRFYZeplqNTQG"> <div class="action-panel-control-wrap"> <label for="rss-name" class="action-panel-label required-field-asterisk"> Name of RSS Feed: </label> <input maxlength="200" placeholder="Name" type="text" name="rss-name" id="rss-name" class="rss-name" value='' required pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/"> </div> <div class="rss-limit-wrap"> <div class="action-panel-control-wrap action-panel-extra-margin-top"> <label for="rss-limit" class="action-panel-label"> Number of items displayed: </label> <select id="rss-limit" class="no-border-panel-selector rss-limit"> <option value="5">5</option> <option value="10">10</option> <option value="15" selected="selected">15</option> <option value="20">20</option> <option value="50">50</option> <option value="100">100</option> </select> </div> </div> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Creating..." data-ga-category="save_share" data-ga-action="alert" data-ga-label="save"> Create RSS </button> <button class="action-panel-cancel" aria-label="Close 'Your RSS' panel" ref="linksrc=close_rss_panel" aria-controls="rss-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="alert" data-ga-label="cancel"> Cancel </button> </div> <div class="action-panel-control-wrap rss-link-copy-wrap"> <label for="rss-link" class="usa-sr-only">RSS Link</label> <input placeholder="Your RSS Feed Link" type="text" name="rss-link" id="rss-link" class="rss-link" title="RSS Link"> <button type="button" disabled class="rss-link-copy-button disabled" data-ga-category="save_share" data-ga-action="rss" data-ga-label="copy"> Copy </button> </div> </form> </div> </div> </div> </div> <div class="article-page" id="article-page" data-article-pmid="19627254"> <aside class="page-sidebar"> <div class="inner-wrap"> <div class="full-text-links"> <div class="full-view"> <h3 class="title"> Full text links </h3> <div class="full-text-links-list"> <a 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class="action-panel-control-wrap new-collections-controls"><label for="collections-action-dialog-add-to-new" class="action-panel-label required-field-asterisk"> Name your collection: </label><input type="text" name="add-to-new-collection" id="collections-action-dialog-add-to-new" class="collections-action-add-to-new" pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/" maxlength="100" data-ga-category="collections_button" data-ga-action="create_collection" data-ga-label="non_favorties_collection"><div class="collections-new-name-too-long usa-input-error-message selection-validation-message"> Name must be less than 100 characters </div></div><div class="action-panel-control-wrap existing-collections-controls"><label for="collections-action-dialog-add-to-existing" class="action-panel-label"> Choose a collection: </label><select id="collections-action-dialog-add-to-existing" class="action-panel-selector 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'Favorites' : 'non_favorites_collection'"></select><div class="collections-retry-load-on-error usa-input-error-message selection-validation-message"> Unable to load your collection due to an error<br><a href="#">Please try again</a></div></div></div><div class="action-panel-actions"><button class="action-panel-submit" type="submit" data-loading-label="Adding..." data-pinger-ignore data-ga-category="collections_button" data-ga-action="click" data-ga-label="add"> Add </button><button class="action-panel-cancel" aria-label="Close 'Add to Collections' panel" ref="linksrc=close_collections_panel" aria-controls="collections-action-panel" aria-expanded="false" data-ga-category="collections_button" data-ga-action="click" data-ga-label="cancel"> Cancel </button></div></form></div></div></div></div><button class="more-actions-button more-actions-dialog-trigger" title="Open dialog with more actions to take" ref="linksrc=more_actions_btn"></button><div class="display-options" 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href="https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22Biochem+J%22%5BTitle+Abbreviation%5D" data-ga-category="search_catalog" data-ga-action="journal_link" data-ga-label="Biochem J"> Search in NLM Catalog </a></li><li><a class="add-to-search-link dropdown-block-link" ref ="linksrc=add_to_search_link" role="button" data-search-term='"Biochem J"[jour]' data-ga-category="search" data-ga-action="add_to_search" data-ga-label='"Biochem J"[jour]' href="#"> Add to Search </a></li></ul></div></div></div><span class="period">. </span><span class="cit">2009 Sep 14;423(1):79-89.</span> </div> <span class="citation-doi"> doi: 10.1042/BJ20090993. </span> </div> <h1 class="heading-title"> Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblasts </h1> <div class="inline-authors"> <div class="authors"> <div class="authors-list"> <span class="authors-list-item "><a class="full-name" href="/?term=Chen+Q&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Qiuyun Chen">Qiuyun Chen</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Department of Molecular Cardiology, Lerner Research Institute, Cleveland Clinic, OH 44195, USA. chenq3@ccf.org" href="#full-view-affiliation-1" ref="linksrc=author_aff"> 1 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Zhang+T&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Teng Zhang">Teng Zhang</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Roshetsky+JF&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Joseph F Roshetsky">Joseph F Roshetsky</a><span 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data-ga-category="search" data-ga-action="author_link" data-ga-label="Qing Wang">Qing Wang</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Hinek+A&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Aleksander Hinek">Aleksander Hinek</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Plow+EF&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Edward F Plow">Edward F Plow</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Dicorleto+PE&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Paul E Dicorleto">Paul E Dicorleto</a></span> </div> </div> </div> <div class="short-article-details"> Affiliations <button class="more-details" id="toggle-authors" data-alt-text="Collapse" aria-controls="expanded-authors" aria-expanded="false" data-pinger-ignore data-fetch-url="/19627254/long-authors/" data-id-prefix=""> <span class="title"> Expand </span> </button> </div> <div class="extended-article-details" id="expanded-authors" aria-hidden="true"> <div class="expanded-authors" id="full-view-expanded-authors"> <div class="affiliations"> <h3 class="title"> Affiliation </h3> <ul class="item-list"> <li data-affiliation-id="full-view-affiliation-1" id="full-view-affiliation-1" ><sup class="key">1</sup> Department of Molecular Cardiology, Lerner Research Institute, Cleveland Clinic, OH 44195, USA. chenq3@ccf.org</li> </ul> </div> </div> </div> <ul class="identifiers" id="full-view-identifiers"> <li> <span class="identifier pubmed"> <span class="id-label"> PMID: </span> <strong class="current-id" title="PubMed ID">19627254</strong> </span> </li> <li> <span class="identifier pmc"> <span class="id-label"> PMCID: </span> <a class="id-link" target="_blank" rel="noopener" ref="linksrc=article_id_link&amp;article_id=PMC3024593&amp;id_type=PMC" href="https://pmc.ncbi.nlm.nih.gov/articles/PMC3024593/" data-ga-category="full_text" data-ga-action="PMCID" > PMC3024593 </a> </span> </li> <li> <span class="identifier doi"> <span class="id-label"> DOI: </span> <a class="id-link" target="_blank" rel="noopener" ref="linksrc=article_id_link&amp;article_id=10.1042/BJ20090993&amp;id_type=DOI" href="https://doi.org/10.1042/bj20090993" data-ga-category="full_text" data-ga-action="DOI" > 10.1042/BJ20090993 </a> </span> </li> </ul> <div class="in-clipboard-label " hidden> Item in Clipboard </div> </div> <div class="short-view" id="short-view-heading"> <h1 class="heading-title"> Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblasts </h1> <div class="short-citation"> <span class="authors-list"> <span class="authors-list-item "><span class="full-name">Qiuyun Chen</span><span class="citation-separator">&nbsp;et al.</span></span> </span> <span class="citation-journal"> Biochem J<span class="citation-separator">.</span> </span> <span class="date"> <time class="citation-year" datetime="2009">2009</time><span class="citation-separator">.</span> </span> </div> <div class="more-article-details-section with-display-settings"> <button class="more-article-details" id="toggle-article-details" data-alt-text="Hide details" ref="linksrc=toggle_article_details" aria-controls="full-article-details" aria-expanded="false" data-fetch-url="/19627254/long-authors/" data-id-prefix=""> <span class="title">Show details</span> </button> <div class="display-options" data-format-key="format" data-default-format-value="abstract" data-current-format-value="abstract" role="region"> <button class="trigger usa-button" title="Change format" data-ga-category="display_options" data-ga-action="display_options_dialog"> <span 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class="add-to-search-link dropdown-block-link" ref ="linksrc=add_to_search_link" role="button" data-search-term='"Biochem J"[jour]' data-ga-category="search" data-ga-action="add_to_search" data-ga-label='"Biochem J"[jour]' href="#"> Add to Search </a></li></ul></div></div></div><span class="period">. </span><span class="cit">2009 Sep 14;423(1):79-89.</span> </div> <span class="citation-doi"> doi: 10.1042/BJ20090993. </span> </div> <div class="expanded-authors" id="short-view-expanded-authors"> <div class="authors"> <h3 class="title"> Authors </h3> <div class="authors-list"> <span class="authors-list-item "><a class="full-name" href="/?term=Chen+Q&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Qiuyun Chen">Qiuyun Chen</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Department of Molecular Cardiology, Lerner Research Institute, Cleveland Clinic, OH 44195, USA. chenq3@ccf.org" href="#short-view-affiliation-1" ref="linksrc=author_aff"> 1 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Zhang+T&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Teng Zhang">Teng Zhang</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Roshetsky+JF&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Joseph F Roshetsky">Joseph F Roshetsky</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Ouyang+Z&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Zhufeng Ouyang">Zhufeng Ouyang</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Essers+J&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Jeroen Essers">Jeroen Essers</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Fan+C&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Chun Fan">Chun Fan</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Wang+Q&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Qing Wang">Qing Wang</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Hinek+A&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Aleksander Hinek">Aleksander Hinek</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Plow+EF&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Edward F Plow">Edward F Plow</a><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Dicorleto+PE&amp;cauthor_id=19627254" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Paul E Dicorleto">Paul E Dicorleto</a></span> </div> </div> <div class="affiliations"> <h3 class="title"> Affiliation </h3> <ul class="item-list"> <li data-affiliation-id="short-view-affiliation-1" id="short-view-affiliation-1" ><sup class="key">1</sup> Department of Molecular Cardiology, Lerner Research Institute, Cleveland Clinic, OH 44195, USA. chenq3@ccf.org</li> </ul> </div> </div> <ul class="identifiers" id="short-view-identifiers"> <li> <span class="identifier pubmed"> <span class="id-label"> PMID: </span> <strong class="current-id" title="PubMed ID">19627254</strong> </span> </li> <li> <span class="identifier pmc"> <span class="id-label"> PMCID: </span> <a class="id-link" target="_blank" rel="noopener" ref="linksrc=article_id_link&amp;article_id=PMC3024593&amp;id_type=PMC" href="https://pmc.ncbi.nlm.nih.gov/articles/PMC3024593/" data-ga-category="full_text" data-ga-action="PMCID" > PMC3024593 </a> </span> </li> <li> <span class="identifier doi"> <span class="id-label"> DOI: </span> <a class="id-link" target="_blank" rel="noopener" ref="linksrc=article_id_link&amp;article_id=10.1042/BJ20090993&amp;id_type=DOI" href="https://doi.org/10.1042/bj20090993" data-ga-category="full_text" data-ga-action="DOI" > 10.1042/BJ20090993 </a> </span> </li> </ul> <div class="in-clipboard-label " hidden> Item in Clipboard </div> </div> </div> </header> <div class="actions-buttons inline"><div class="inner-wrap"><button class="full-text-links-button full-text-links-dialog-trigger" title="Open dialog with full text links" ref="linksrc=full_text_links"> Full text links </button><button class="citation-button citation-dialog-trigger" aria-label="Open dialog with citation text in different styles" data-ga-category="save_share" data-ga-action="cite" data-ga-label="open" data-all-citations-url="/19627254/citations/" data-citation-style="nlm" data-pubmed-format-link="/19627254/export/"><span class="button-label">Cite</span></button><button class="more-actions-button more-actions-dialog-trigger" title="Open dialog with more actions to take" ref="linksrc=more_actions_btn"></button><div class="display-options" data-format-key="format" data-default-format-value="abstract" data-current-format-value="abstract" role="region"><button class="trigger usa-button" title="Change format" data-ga-category="display_options" data-ga-action="display_options_dialog"><span class="button-label">Display options</span></button><div class="dropdown" hidden><div class="title"> Display options </div><div class="content"><div class="format-container"><span class="option-label"> Format </span><select name="format" data-initial-value="abstract" aria-label="Display format" id="inline-actions-display-format"><option value="abstract">Abstract</option><option value="pubmed">PubMed</option><option value="pmid">PMID</option></select></div></div></div></div></div></div> <div class="abstract" id="abstract"> <h2 class="title"> Abstract </h2> <div class="abstract-content selected" id="eng-abstract"> <p> Elastic fibres are essential for normal physiology in numerous tissues, including arteries, lungs and skin. Fibulin-4 is an elastic-fibre-associated glycoprotein that is indispensable for elastic-fibre formation in mice. However, the mechanism by which fibulin-4 executes this function remains to be determined. Here, we established an in vitro functional assay system in which fibulin-4 was knocked down in human foreskin fibroblasts using siRNA (small interfering RNA) technology. With two different siRNAs, substantial knockdown of fibulin-4 was achieved, and this suppression was associated with impaired elastic-fibre formation by the fibroblasts. Real-time reverse transcription-PCR analysis showed that knockdown of fibulin-4 expression was accompanied by reduced expression of tropoelastin mRNA. Further analysis showed that this decrease was caused by transcriptional down-regulation of tropoelastin. This effect was selective, since the mRNA level of other elastic-fibre-associated proteins, including fibrillin-1, lysyl oxidase and lysyl oxidase-like-1, was not affected. Moreover, addition of conditioned medium from cultures of CHO (Chinese-hamster ovary) cells overexpressing fibulin-4 stimulated tropoelastin expression and elastic-fibre formation in cultures of Williams-Beuren-syndrome fibroblasts. Knocking down or knocking out fibulin-4 in mice led to a decrease in tropoelastin expression in the aorta. These results indicate that fibulin-4, considered as a structural protein, may also participate in regulating elastic-fibre formation in human cells through an unanticipated mechanism, namely the regulation of tropoelastin expression. </p> </div> </div> <p class="disclaimer" id="disclaimer"> <a href="/disclaimer/" target="_blank" data-ga-category="literature_resources" data-ga-action="disclaimer_link" data-ga-label="under_abstract">PubMed Disclaimer</a> </p> <div class="figures" id="figures"> <h2 class="title"> Figures </h2> <div class="figures-list" id="slides-container" itemscope itemtype="http://schema.org/ImageGallery"> <figure class="figure-item " itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="0" data-label-slug="figure-1"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/7058c0d52568/nihms261918f1.jpg" itemprop="contentUrl" aria-describedby="figure-caption-0" role="button" data-image-width="800" data-image-height="71" data-image-alt="Figure 1" data-pmc-id="PMC3024593" data-figure-id="F1"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-0" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/c7c278ccfadb/nihms261918f1.gif" alt="Figure 1" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="800"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="71"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 1. Partial amino acid sequence of… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 1. Partial amino acid sequence of the N-terminal His <sub> 6 </sub> –c-myc-tagged human fibulin-4 expressed… </p> </strong> </div> <figcaption id="figure-caption-0" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 1. Partial amino acid sequence of the N-terminal His<sub>6</sub>–c-myc-tagged human fibulin-4 expressed by plasmid pQC0701 </strong> <div class="figure-caption-contents">The expressed protein contains an N-terminal Ig<i>κ</i>-chain signal peptide (underlined sequence). The predicted signal peptide cleavage site is indicated by a filled triangle. The N-terminus of the cleaved polypeptide has a His<sub>6</sub> tag and a c-myc epitope tag (italicized and bold). The predicted mature form of human fibulin-4 polypeptide was placed C-terminal to the myc epitope in parenthesis. Only eight amino acid residues from both the N-terminus and the C-terminus of human fibulin-4 are shown here.</div> </figcaption> </figure> <figure class="figure-item " itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="1" data-label-slug="figure-2"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/39d8770cff64/nihms261918f2.jpg" itemprop="contentUrl" aria-describedby="figure-caption-1" role="button" data-image-width="506" data-image-height="720" data-image-alt="Figure 2" data-pmc-id="PMC3024593" data-figure-id="F2"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-1" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/2c8097150ffc/nihms261918f2.gif" alt="Figure 2" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="506"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="720"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 2. Relative mRNA abundance in fibroblasts… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 2. Relative mRNA abundance in fibroblasts infected with control pLK viruses or fibulin-4 shRNA… </p> </strong> </div> <figcaption id="figure-caption-1" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 2. Relative mRNA abundance in fibroblasts infected with control pLK viruses or fibulin-4 shRNA viruses pL335 and pL337 </strong> <div class="figure-caption-contents">Infecting HFFs with pL335 or pL337 viruses leads to a significant decrease in fibulin-4 mRNA (<b>A</b>), with no statistically significant change in the mRNA level (<b>B</b>) of fibrillin-1 (hFBN1), lysyl oxidase (hLOX), and lysyl oxidase-like-1 (hLOXL1). Results are means ± S.D. for six to eight samples. **<i>P &lt;</i> 0.05 compared with the control pLK.</div> </figcaption> </figure> <figure class="figure-item " itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="2" data-label-slug="figure-3"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/224be2d8dcc1/nihms261918f3.jpg" itemprop="contentUrl" aria-describedby="figure-caption-2" role="button" data-image-width="506" data-image-height="750" data-image-alt="Figure 3" data-pmc-id="PMC3024593" data-figure-id="F3"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-2" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/9d78b698818a/nihms261918f3.gif" alt="Figure 3" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="506"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="750"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 3. Characterization of fibulin-4 produced in… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 3. Characterization of fibulin-4 produced in HFF cells </p> </strong> <div class="figure-caption-contents"><p> ( <b> A </b> ) Western-blot analysis of… </p></div> </div> <figcaption id="figure-caption-2" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 3. Characterization of fibulin-4 produced in HFF cells </strong> <div class="figure-caption-contents">(<b>A</b>) Western-blot analysis of fibulin-4 in HFF cell lysate and cultured medium. (<b>B</b>–<b>G</b>) Confocal images of passage-10 HFF cultures immunostained with antibodies against human fibulin-4 (<b>B</b>, <b>E</b>), human elastin (<b>C</b>) and human fibrillin-1 (<b>F</b>). Co-localization of fibulin-4 with elastin and fibrillin-1 are shown in the merged images, (<b>D</b>) and (<b>G</b>) respectively. (<b>H</b>, <b>I</b>) Fluorescent images of HFF cultures infected with pLK virus (<b>H</b>) or pL337 virus (<b>I</b>) and immunostained with fibulin-4 antibody. (<b>J</b>) Western-blot analysis of fibulin-4 (FB4) in HFF cells infected with the control pLK lentivirus or the fibulin-4 shRNA-producing lentivirus pL337. Note that pL337 infection led to the abrogation of fibulin-4 staining in the ECM (<b>I</b>) and a significant decrease in the amount of fibulin-4 in the HFF cells (<b>J</b>).</div> </figcaption> </figure> <figure class="figure-item " itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="3" data-label-slug="figure-4"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/24b21b3912e8/nihms261918f4.jpg" itemprop="contentUrl" aria-describedby="figure-caption-3" role="button" data-image-width="509" data-image-height="925" data-image-alt="Figure 4" data-pmc-id="PMC3024593" data-figure-id="F4"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-3" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/ca43692de3ab/nihms261918f4.gif" alt="Figure 4" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="509"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="925"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 4. Representative images of HFFs immunostained… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 4. Representative images of HFFs immunostained with antibodies against elastin (A, B and C)… </p> </strong> </div> <figcaption id="figure-caption-3" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 4. Representative images of HFFs immunostained with antibodies against elastin (A, B and C) or fibrillin-1 (D, E and F) and HPCE cells immunostained with antibodies against fibulin-4 (I, K), elastin (J) or fibrillin-1 (L) </strong> <div class="figure-caption-contents">HFF cells were seeded on to glass coverslips in a 12-well plate with 1.8 × 10<sup>5</sup> cells/well and were allowed to grow for 3 days before immunostaining. Fibroblasts infected with pLK control viruses (A) produced extensive elastic fibres, whereas fibroblasts infected with fibulin-4 shRNA viruses pL335 (B) and pL337(C) produced significantly fewer elastic fibres. No statistically significant difference in the production of fibrillin-1 microfibrils was present among fibroblast cultures infected with pLK (D), pL335 (E) and pL337 (F). (G) Quantification of elastic fibres taken from at least four fields of multiple cultures represented in (A), (B) and (C). (H) Quantification of microfibrils taken from four fields of multiple cultures represented in (D), (E) and (F). **<i>P &lt;</i> 0.05 compared with the respective control pLK. (<b>I</b>–<b>L</b>) HPCE cells were seeded on to glass coverslips in a 12-well plate with 4 × 10<sup>5</sup> cells/well and were allowed to grow for 7 days before immunostaining. The cultures produced significant amounts of fibulin-4 (<b>I</b>, <b>K</b>) and fibrillin-1 (<b>L</b>). However, no elastic fibres were detectable (<b>J</b>).</div> </figcaption> </figure> <figure class="figure-item " itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="4" data-label-slug="figure-5"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/dfd4cd541c19/nihms261918f5.jpg" itemprop="contentUrl" aria-describedby="figure-caption-4" role="button" data-image-width="800" data-image-height="506" data-image-alt="Figure 5" data-pmc-id="PMC3024593" data-figure-id="F5"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-4" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/86fe1437a9b6/nihms261918f5.gif" alt="Figure 5" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="800"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="506"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 5. Relative tropoelastin mRNA abundance in… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 5. Relative tropoelastin mRNA abundance in fibroblasts infected with control pLK viruses or fibulin-4… </p> </strong> </div> <figcaption id="figure-caption-4" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 5. Relative tropoelastin mRNA abundance in fibroblasts infected with control pLK viruses or fibulin-4 shRNA viruses pL335 and pL337 </strong> <div class="figure-caption-contents">Infecting HFFs with either pL335 or pL337 viruses led to a significant decrease in tropoelastin mRNA. Results are means±S.D. for six to eight samples. **<i>P &lt;</i> 0.05 compared with the control pLK. The results are based on eight samples from three experiments. Some variability in the magnitude of tropoelastin mRNA expression was observed and later traced to the batches of FBS used.</div> </figcaption> </figure> <figure class="figure-item " itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="5" data-label-slug="figure-6"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/25f1bd37b4db/nihms261918f6.jpg" itemprop="contentUrl" aria-describedby="figure-caption-5" role="button" data-image-width="602" data-image-height="377" data-image-alt="Figure 6" data-pmc-id="PMC3024593" data-figure-id="F6"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-5" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/4fd3c6f4f854/nihms261918f6.gif" alt="Figure 6" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="602"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="377"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 6. Relative tropoelastin mRNA abundance in… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 6. Relative tropoelastin mRNA abundance in rASMCs infected with control pLK viruses or fibulin-4… </p> </strong> </div> <figcaption id="figure-caption-5" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 6. Relative tropoelastin mRNA abundance in rASMCs infected with control pLK viruses or fibulin-4 shRNA virus pL337 </strong> <div class="figure-caption-contents">Infecting rASMCs with pL337 virus leads to a significant decrease in tropoelastin mRNA, as analysed by real-time RT–PCR. Results are means ± S.D. for eight samples. **<i>P &lt;</i> 0.05 compared with the control pLK.</div> </figcaption> </figure> <figure class="figure-item tail" itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="6" data-label-slug="figure-7"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/15c40fc8d968/nihms261918f7.jpg" itemprop="contentUrl" aria-describedby="figure-caption-6" role="button" data-image-width="510" data-image-height="603" data-image-alt="Figure 7" data-pmc-id="PMC3024593" data-figure-id="F7"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-6" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/cf9e3c8add62/nihms261918f7.gif" alt="Figure 7" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="510"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="603"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 7. Decreased tropoelastin expression in the… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 7. Decreased tropoelastin expression in the aorta of fibulin-4-knockdown and fibulin-4-knockout mice </p> </strong> <div class="figure-caption-contents"><p> ( <b> A… </b> </p></div> </div> <figcaption id="figure-caption-6" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 7. Decreased tropoelastin expression in the aorta of fibulin-4-knockdown and fibulin-4-knockout mice </strong> <div class="figure-caption-contents">(<b>A</b>) Real-time RT–PCR analysis of fibulin-4 (mFbln4) and tropoelastin (mEln) mRNA in the aorta of 10-day-old fibulin-4-knock-down mice. Results are means ± S.E.M. for nine to eleven samples. **<i>P &lt;</i> 0.05 compared with the wild-type (wt) littermates. het, heterozygous; homo, homozygous. (<b>B</b>) Immunostaining images of E13.5 aorta isolated from fibulin-4-knockout mice. Top panel, stained with tropoelastin antibodies; Bottom panel, stained with control rabbit serum. The experiment was repeated at least three times with consistent results.</div> </figcaption> </figure> <figure class="figure-item tail" itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="7" data-label-slug="figure-8"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/e33e847ddba4/nihms261918f8.jpg" itemprop="contentUrl" aria-describedby="figure-caption-7" role="button" data-image-width="641" data-image-height="484" data-image-alt="Figure 8" data-pmc-id="PMC3024593" data-figure-id="F8"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-7" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/effdabc2c1eb/nihms261918f8.gif" alt="Figure 8" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="641"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="484"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 8. Changes over time of relative… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 8. Changes over time of relative tropoelastin mRNA abundance in fibroblasts infected with control… </p> </strong> </div> <figcaption id="figure-caption-7" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 8. Changes over time of relative tropoelastin mRNA abundance in fibroblasts infected with control pLK virus or fibulin-4 shRNA viruses pL335 and pL337 </strong> <div class="figure-caption-contents">The rate of tropoelastin mRNA degradation in pL335- or pL337-infected fibroblasts was not significantly different from that in pLK-infected cells as determined by Student’s <i>t</i> test. Results are means ± S.D. for five to eight samples.</div> </figcaption> </figure> <figure class="figure-item tail" itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="8" data-label-slug="figure-9"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/df8b62506b40/nihms261918f9.jpg" itemprop="contentUrl" aria-describedby="figure-caption-8" role="button" data-image-width="800" data-image-height="523" data-image-alt="Figure 9" data-pmc-id="PMC3024593" data-figure-id="F9"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-8" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/a7c6dd5c4a22/nihms261918f9.gif" alt="Figure 9" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="800"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="523"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 9. Relative amount of tropoelastin pre-mRNA… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 9. Relative amount of tropoelastin pre-mRNA in fibroblasts infected with control pLK viruses or… </p> </strong> </div> <figcaption id="figure-caption-8" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 9. Relative amount of tropoelastin pre-mRNA in fibroblasts infected with control pLK viruses or fibulin-4 shRNA viruses pL335 and pL337 </strong> <div class="figure-caption-contents">Fibroblasts infected with pL335 or pL337 produced a significant decreased amount of tropoelastin pre-mRNA compared with that in the pLK-infected cells, indicating that the transcriptional activity of tropoelastin significantly decreased in the pL335- or pL337-infected cells as compared with that in the pLK-infected cells. Results are means ± S.D. for four or five samples. **<i>P &lt;</i> 0.05 compared with the control pLK.</div> </figcaption> </figure> <figure class="figure-item tail" itemscope itemtype="http://schema.org/ImageObject" itemprop="associatedMedia" data-slide-index="9" data-label-slug="figure-10"> <a class="figure-link" href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/5aba07eafe4f/nihms261918f10.jpg" itemprop="contentUrl" aria-describedby="figure-caption-9" role="button" data-image-width="604" data-image-height="507" data-image-alt="Figure 10" data-pmc-id="PMC3024593" data-figure-id="F10"> <img class="figure-thumb" itemprop="thumbnail" id="article-image-9" src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32a0/3024593/16a5638a5bbd/nihms261918f10.gif" alt="Figure 10" /> </a> <meta itemprop="width" itemtype="http://schema.org/ImageObject" content="604"> <meta itemprop="height" itemtype="http://schema.org/ImageObject" content="507"> <div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true"> <div class="caption-wrap"> <strong class="figure-label"> <p> Figure 10. Effects of fibulin-4 on the… </p> </strong> </div> </div> <div class="figure-caption-medium figure-caption-text" aria-hidden="true"> <strong class="figure-label"> <p> Figure 10. Effects of fibulin-4 on the elastogenesis of WBS fibroblasts </p> </strong> <div class="figure-caption-contents"><p> ( <b> A </b> ) Western-blot… </p></div> </div> <figcaption id="figure-caption-9" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description"> <strong class="figure-label"> Figure 10. Effects of fibulin-4 on the elastogenesis of WBS fibroblasts </strong> <div class="figure-caption-contents">(<b>A</b>) Western-blot analysis of fibulin-4 in CHO-cell conditioned medium. Fibulin-4 was detected only in the conditioned medium (lane FB4) of fibulin-4-overexpressing cells and not in the conditioned medium (lane C) of control cells. (<b>B</b>, <b>C</b>) Expression of tropoelastin and <i>GAPDH</i> mRNAs in the WBS fibroblast cultures in DMEM with 2 % FBS (lane 1), in conditioned medium from control CHO cells mixed with equal volume of DMEM with 2 % FBS (lane 2) or in conditioned medium from CHO cells overexpressing fibulin-4 cells mixed with equal volume of DMEM with 2 % FBS (lane 3). Fibroblasts exposed to fibulin-4-overexpressing conditioned medium demonstrated a significantly higher level of tropoelastin mRNA. The experiment was repeated three times. **<i>P &lt;</i> 0.0001 compared with lane 1 or lane 2. (<b>D</b>) WBS fibroblasts maintained for 5 days either in DMEM containing 2 % FBS or in the medium consisting of equal volumes of DMEM and conditioned medium from control CHO cell cultures did not form elastic fibres (<b>D</b>, panel 1) and (<b>D</b>, panel 2). In contrast, the parallel cultures of WBS fibroblasts maintained in medium consisting of equal volumes of DMEM and conditioned medium from cultures of CHO cells overexpressing fibulin-4 (final concn. of fibulin-4: 10 ng/ml) demonstrated a significant formation of immunodetectable elastic fibres (<b>D</b>, panel 3).</div> </figcaption> </figure> </div> <div class="see-all actions-bar"> <button class="show-more" aria-controls="slides-container" data-ga-category="figure" data-ga-action="show_all"> All figures (10) </button> </div> <!-- Root element of PhotoSwipe. Must have class pswp. --> <div class="pswp figures-dialog" id="figures-dialog" tabindex="-1" role="dialog" aria-hidden="true" data-image-gallery-uid="article-figures" aria-label="Figures Dialog"> <!-- Background of PhotoSwipe. 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Cell Adhes Commun. 1994;2:185–193. - <a class="reference-link" href="/7827955/" ref="linksrc=references_link&amp;ordinalpos=1" data-ga-category="reference" data-ga-action="7827955" > PubMed </a> </li> </ol> </li> </ol> <div class="actions-bar"> <button class="show-all" ref="linksrc=show_all_references_link" aria-controls="top-references-list-1" data-next-page-url="/19627254/references/" data-ga-category="reference" data-ga-action="show_more" > Show all 64 references </button> </div> </div> </div> </div> <div id="publication-types" class="publication-types keywords-section"> <h2 class="title"> Publication types </h2> <ul class="keywords-list"><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link" aria-label="Toggle dropdown menu for keyword Research Support, N.I.H., Extramural" tabindex="0" aria-controls="keyword-actions-publication-types-0" aria-expanded="false" data-pinger-ignore> Research Support, N.I.H., Extramural </button><div 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