Phenotypic Alteration of Vascular Smooth Muscle Cells Precedes Elastolysis in a Mouse Model of Marfan Syndrome

ABSTRACT—Marfan syndrome is associated with early death due to aortic aneurysm. The condition is caused by mutations in the gene (FBN1) encoding fibrillin-1, a major constituent of extracellular microfibrils. Prior observations suggested that a deficiency of microfibrils causes failure of elastic fi...

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Veröffentlicht in:	Circulation research 2001-01, Vol.88 (1), p.37-43
Hauptverfasser:	Bunton, Tracie E, Jensen Biery, Nancy, Myers, Loretha, Gayraud, Barbara, Ramirez, Francesco, Dietz, Harry C
Format:	Artikel
Sprache:	eng
Schlagworte:	Actins - analysis Adolescent Adult Animals Aorta - metabolism Aorta - pathology Aorta - ultrastructure Biological and medical sciences Disease Models, Animal Elastic Tissue - pathology Fibrillin-1 Fibrillins Humans Immunohistochemistry In Situ Hybridization Marfan Syndrome - metabolism Marfan Syndrome - pathology Matrix Metalloproteinase 9 - analysis Medical sciences Mice Mice, Knockout Microfibrils - metabolism Microfilament Proteins - deficiency Microfilament Proteins - genetics Microscopy, Electron Middle Aged Muscle, Smooth, Vascular - chemistry Muscle, Smooth, Vascular - metabolism Muscle, Smooth, Vascular - pathology Phenotype RNA, Messenger - genetics RNA, Messenger - metabolism Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Tropoelastin - genetics Tropoelastin - metabolism Vimentin - analysis
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