Targeting Transforming Growth Factor-β Signaling in Aortopathies in Marfan Syndrome
نویسندگان
چکیده
منابع مشابه
Targeting transforming growth factor-β signaling in aortopathies in Marfan syndrome.
topathy, with initial experiments showing prevention of aortic root expansion in a mouse model of MFS using RAS blockade,3 which was followed by studies in human patients that showed similar results.4,5 These findings were welcomed with enthusiasm as a pharmacological solution for MFS, and have prompted several human clinical trials, which are presently ongoing to determine the possible benefit...
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TGF-β pathway is being extensively evaluated as a potential therapeutic target. The transforming growth factor-β (TGF-β) signaling pathway has the dual role in both tumor suppression and tumor promotion. To design cancer therapeutics successfully, it is important to understand TGF-β related functional contexts. This review discusses the molecular mechanism of the TGF-β pathway and describes the...
متن کاملThe role of transforming growth factor-beta in Marfan syndrome.
The starting point, in Marfan syndrome (MFS) appears to be the mutation of fibrillin-1 gene whose deconstructed protein product cannot bind transforming growth factor beta (TGF-b), leading to an increased TGF-b tissue level. The aim of this review is to review the already known features of the cellular signal transduction downstream to TGF-b and its impact on the tissue homeostasis of microfibr...
متن کاملAortopathy in a Mouse Model of Marfan Syndrome Is Not Mediated by Altered Transforming Growth Factor β Signaling
BACKGROUND Marfan syndrome (MFS) is caused by mutations in the gene encoding fibrillin-1 (FBN1); however, the mechanisms through which fibrillin-1 deficiency causes MFS-associated aortopathy are uncertain. Recently, attention was focused on the hypothesis that MFS-associated aortopathy is caused by increased transforming growth factor-β (TGF-β) signaling in aortic medial smooth muscle cells (SM...
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3 Chen Zhang, Chek Kun Tan, Craig McFarlane, Mridula Sharma, Nguan Soon Tan*, Ravi 4 Kambadur,*# 5 1. School of Biological Sciences, Nanyang Technological University, Singapore 637551. 6 2 Singapore Institute for Clinical Sciences, A*STAR, 30 Medical Centre Drive, National 7 University of Singapore, Singapore 8 3 Department of Biochemistry, YLL School of Medicine, National University of 9 Singa...
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ژورنال
عنوان ژورنال: Circulation Journal
سال: 2013
ISSN: 1346-9843,1347-4820
DOI: 10.1253/circj.cj-13-0183