Differences in genetic signaling, and not mechanical properties of the wall, are linked to ascending aortic aneurysms in fibulin-4 knockout mice

Fibulin-4 is an extracellular matrix protein that is essential for proper assembly of arterial elastic fibers. Mutations in fibulin-4 cause cutis laxa with thoracic aortic aneurysms (TAAs). Sixty percent of TAAs occur in the ascending aorta (AA). Newborn mice lacking fibulin-4 (Fbln4(-/-)) have aneu...

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Veröffentlicht in:	American journal of physiology. Heart and circulatory physiology 2015-07, Vol.309 (1), p.H103-H113
Hauptverfasser:	Kim, Jungsil, Procknow, Jesse D, Yanagisawa, Hiromi, Wagenseil, Jessica E
Format:	Artikel
Sprache:	eng
Schlagworte:	Acute-Phase Proteins - genetics Acute-Phase Proteins - metabolism Aneurysms Animals Animals, Newborn Aorta - metabolism Aorta - physiopathology Aorta - ultrastructure Aorta, Thoracic - metabolism Aorta, Thoracic - physiopathology Aorta, Thoracic - ultrastructure Aortic Aneurysm, Thoracic - genetics Calcium-Binding Proteins Collagen Type VIII - genetics Collagen Type VIII - metabolism Cyclooxygenase 2 - genetics Cyclooxygenase 2 - metabolism Elastic Modulus Epiregulin - genetics Epiregulin - metabolism Extracellular Matrix Proteins - genetics Gene expression Gene Expression Profiling Genotype & phenotype Heparin-binding EGF-like Growth Factor - genetics Heparin-binding EGF-like Growth Factor - metabolism Matrix Metalloproteinase 8 - genetics Matrix Metalloproteinase 8 - metabolism Mice Mice, Knockout Microscopy, Electron Muscle Proteins - genetics Muscle Proteins - metabolism Muscle, Smooth, Vascular - cytology Muscle, Smooth, Vascular - metabolism Mutation Myocytes, Smooth Muscle - metabolism Myocytes, Smooth Muscle - ultrastructure Nuclear Proteins - genetics Nuclear Proteins - metabolism Pathogenesis Physiology Proteins Receptors, Cell Surface - genetics Receptors, Cell Surface - metabolism Receptors, G-Protein-Coupled Repressor Proteins - genetics Repressor Proteins - metabolism Reverse Transcriptase Polymerase Chain Reaction RNA, Messenger - metabolism Serpins - genetics Serpins - metabolism Up-Regulation Vascular Biology and Microcirculation
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