Fibroblast-Secreted Phosphoprotein 1 Mediates Extracellular Matrix Deposition and Inhibits Smooth Muscle Cell Contractility in Marfan Syndrome Aortic Aneurysm

Fibrillin 1 (Fbn1) mutation causes Marfan syndrome (MFS) with thoracic aortic aneurysm (TAA) as the main complication. The mechanisms for extracellular matrix (ECM) homeostasis disruption in MFS TAA are unclear. Here, we found ECM-related gene secreted phosphoprotein 1 (Spp1) increased in Fbn1 C1041...

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Veröffentlicht in:	Journal of cardiovascular translational research 2022-10, Vol.15 (5), p.959-970
Hauptverfasser:	Zhou, Mei, Zhu, Yuexin, Zhou, Zeyi, Qi, Feiran, Zheng, Shuai, Gao, Shijuan, Li, Yulin, Liu, Yan, Du, Jie
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Aortic Aneurysm - genetics Aortic Aneurysm, Thoracic - genetics Biomedical Engineering and Bioengineering Biomedicine Cardiology Extracellular Matrix - metabolism Fibroblasts - metabolism Human Genetics Marfan Syndrome - complications Marfan Syndrome - genetics Marfan Syndrome - metabolism Medicine Medicine & Public Health Mice Muscle, Smooth, Vascular - metabolism Myocytes, Smooth Muscle - metabolism Original Article Osteopontin - genetics Osteopontin - metabolism
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