{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T22:42:19Z","timestamp":1791758539866,"version":"4.3.4"},"reference-count":41,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,9,29]],"date-time":"2014-09-29T00:00:00Z","timestamp":1411948800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NIH","award":["CA034282 AR49698"],"award-info":[{"award-number":["CA034282 AR49698"]}]},{"name":"NSERC RGPIN","award":["355710"],"award-info":[{"award-number":["355710"]}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["3 RO1 CA034282-28S1"],"award-info":[{"award-number":["3 RO1 CA034282-28S1"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal Cellular Physiology"],"published-print":{"date-parts":[[2015,1]]},"abstract":"<jats:sec><jats:label\/><jats:p>Mice deficient in Latent TGF\u03b2 Binding Protein 4 (Ltbp4) display a defect in lung septation and elastogenesis. The lung septation defect is normalized by genetically decreasing TGF\u03b22 levels. However, the elastic fiber assembly is not improved in <jats:italic>Tgfb2<jats:sup>\u2010\/\u2010<\/jats:sup>;Ltbp4S<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> compared to <jats:italic>Ltbp4S<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> lungs. We found that decreased levels of TGF\u03b21 or TGF\u03b23 did not improve lung septation indicating that the TGF\u03b2 isoform elevated in <jats:italic>Ltbp4S<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> lungs is TGF\u03b22. Expression of a form of Ltbp4 that could not bind latent TGF\u03b2 did not affect lung phenotype indicating that normal lung development does not require the formation of LTBP4\u2010latent TGF\u03b2 complexes. Therefore, the change in TGF\u03b2 level in the lungs is not directly related to Ltbp4 deficiency but probably is a consequence of changes in the extracellular matrix. Interestingly, combination of the <jats:italic>Ltbp4S<\/jats:italic><jats:sup>\u2010\/\u2010<\/jats:sup> mutation with a fibulin\u20105 null mutant in F<jats:italic>bln5<jats:sup>\u2010\/\u2010<\/jats:sup>;Ltbp4S<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> mice improves the lung septation compared to <jats:italic>Ltbp4S<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> lungs. Large globular elastin aggregates characteristic for <jats:italic>Ltbp4S<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> lungs do not form in <jats:italic>Fbln5<jats:sup>\u2010\/\u2010<\/jats:sup>;Ltbp4S<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> lungs and EM studies showed that elastic fibers in <jats:italic>Fbln5<\/jats:italic><jats:sup>\u2010\/\u2010<\/jats:sup><jats:italic>;Ltbp4S<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> lungs resemble those found in <jats:italic>Fbln5<jats:sup>\u2010\/\u2010<\/jats:sup><\/jats:italic> mice. These results are consistent with a role for TGF\u03b22 in lung septation and for Ltbp4 in regulating fibulin\u20105 dependent elastic fiber assembly. J. Cell. Physiol. 230: 226\u2013236, 2015. \u00a9 2014 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jcp.24704","type":"journal-article","created":{"date-parts":[[2014,6,24]],"date-time":"2014-06-24T21:19:13Z","timestamp":1403644753000},"page":"226-236","source":"Crossref","is-referenced-by-count":50,"title":["Function of Latent TGF\u03b2 Binding Protein 4 and Fibulin 5 in Elastogenesis and Lung Development"],"prefix":"10.1002","volume":"230","author":[{"given":"Branka","family":"Dabovic","sequence":"first","affiliation":[{"name":"Department of Cell Biology New York University Langone Medical Center New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian B.","family":"Robertson","sequence":"additional","affiliation":[{"name":"Department of Cell Biology New York University Langone Medical Center New York New 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