| Badly engineered fibrillin lessons from molecular studies of marfan syndrome. | |
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MedLine Citation:
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PMID: 21235897 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Marfan syndrome (MFS) is one of the most common inherited connective tissue disorders that severely affects the cardiovascular system. Mutations in the gene encoding fibrillin-1 (FBN1) have been shown to cause MFS as well as dominant ectopia lentis and neonatal Marfan syndrome. Fibrillin-1 is the major component of elastic fiber microfibrils in the extracellular matrix of connective tissue. Recent molecular studies have brought some light into understanding the pathogenesis of MFS, but the diagnostic problems still prevail, and targeted therapy of MFS must await better dissection of the role of fibrillin-1 in tissue phenotype using different experimental systems. (Trends Cardiovasc Med 1997;7:282-288). © 1997, Elsevier Science Inc. |
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Authors:
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T Rantamäki; L Karttunen; L Peltonen |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Trends in cardiovascular medicine Volume: 7 ISSN: 1050-1738 ISO Abbreviation: Trends Cardiovasc. Med. Publication Date: 1997 Nov |
Date Detail:
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Created Date: 2011-01-17 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9108337 Medline TA: Trends Cardiovasc Med Country: United States |
Other Details:
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Languages: eng Pagination: 282-8 Citation Subset: - |
Copyright Information:
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Copyright © 1997 Elsevier Science Inc. All rights reserved. |
Affiliation:
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Department of Human Molecular Genetics, National Public Health Institute, FIN-00300 Helsinki, Finland. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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