| Drug-based therapies for vascular disease in Marfan syndrome: from mouse models to human patients. | |
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MedLine Citation:
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PMID: 20687182 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Marfan syndrome is a congenital disorder of the connective tissue with a long history of clinical and basic science breakthroughs that have forged our understanding of vascular-disease pathogenesis. The biomedical importance of Marfan syndrome was recently underscored by the discovery that the underlying genetic lesion impairs both tissue integrity and transforming growth factor-beta regulation of cell behavior. This discovery has led to the successful implementation of the first pharmacological intervention in a connective-tissue disorder otherwise incurable by either gene-based or stem cell-based therapeutic strategies. More generally, information gathered from the study of Marfan syndrome pathogenesis has the potential to improve the clinical management of common acquired disorders of connective-tissue degeneration. |
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Authors:
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Jason R Cook; Harikiran Nistala; Francesco Ramirez |
Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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Title: The Mount Sinai journal of medicine, New York Volume: 77 ISSN: 1931-7581 ISO Abbreviation: Mt. Sinai J. Med. Publication Date: 2010 Jul-Aug |
Date Detail:
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Created Date: 2010-08-05 Completed Date: 2011-06-02 Revised Date: 2011-08-01 |
Medline Journal Info:
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Nlm Unique ID: 0241032 Medline TA: Mt Sinai J Med Country: United States |
Other Details:
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Languages: eng Pagination: 366-73 Citation Subset: IM |
Copyright Information:
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2010 Mount Sinai School of Medicine. |
Affiliation:
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Mount Sinai School of Medicine, New York, NY, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Angiotensin II Type 1 Receptor Blockers
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therapeutic use Animals Antihypertensive Agents / pharmacology, therapeutic use Aorta / pathology* Aortic Aneurysm, Thoracic / drug therapy*, genetics, pathology Connective Tissue / drug effects, pathology* Disease Models, Animal Humans Losartan / pharmacology, therapeutic use Marfan Syndrome / drug therapy*, genetics, pathology Mice Microfilament Proteins / drug effects Renin-Angiotensin System / drug effects Signal Transduction / drug effects Transforming Growth Factor beta / drug effects |
| Grant Support | |
ID/Acronym/Agency:
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AR-049 698/AR/NIAMS NIH HHS; P01 AR049698-050001/AR/NIAMS NIH HHS; T32GM062754/GM/NIGMS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Angiotensin II Type 1 Receptor Blockers; 0/Antihypertensive Agents; 0/Microfilament Proteins; 0/Transforming Growth Factor beta; 0/fibrillin; 114798-26-4/Losartan |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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