Defective Connective Tissue Remodeling in Smad3 Mice Leads to Accelerated Aneurysmal Growth Through Disturbed Downstream TGF-β Signaling

Defective Connective Tissue Remodeling in Smad3 Mice Leads to Accelerated Aneurysmal Growth Through Disturbed Downstream TGF-β Signaling

Aneurysm-osteoarthritis syndrome characterized by unpredictable aortic aneurysm formation, is caused by SMAD3 mutations. SMAD3 is part of the SMAD2/3/4 transcription factor, essential for TGF-β-activated transcription. Although TGF-β-related gene mutations result in aneurysms, the underlying mechani...

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Bibliographic Details
Main Authors: I. van der Pluijm, PhD, N. van Vliet, BSc, J.H. von der Thusen, PhD, J.L. Robertus, PhD, Y. Ridwan, BSc, P.M. van Heijningen, BSc, B.S. van Thiel, MSc, M. Vermeij, BSc, S.E. Hoeks, PhD, R.M.G.B. Buijs-Offerman, PhD, H.J.M. Verhagen, MD, PhD, R. Kanaar, PhD, A.M. Bertoli-Avella, MD, PhD, J. Essers, PhD
Format: Article
Language:English
Published: Elsevier 2016-10-01
Series:EBioMedicine
Subjects:
Aneurysm
Vascular biology
TGF-β signaling pathway
Echocardiography
Mouse model
Online Access:http://www.sciencedirect.com/science/article/pii/S2352396416304133

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http://www.sciencedirect.com/science/article/pii/S2352396416304133

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