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Abstract
Loeys-Dietz syndrome (LDS) is a connective tissue disorder that is characterized by skeletal abnormalities, craniofacial malformations, and a high predisposition for aortic aneurysm. In this issue of the JCI, Gallo et al. developed transgenic mouse strains harboring missense mutations in the genes encoding type I or II TGF-β receptors. These mice exhibited several LDS-associated phenotypes. Despite being functionally defective, the mutated receptors enhanced TGF-β signaling in vivo, inferred by detection of increased levels of phosphorylated Smad2. Aortic aneurysms in these LDS mice were ablated by treatment with the Ang II type 1 (AT1) receptor antagonist losartan. The results from this study will foster further interest into the potential therapeutic implications of AT1 receptor antagonists.
Document Type
Commentary
Publication Date
1-2-2014
Digital Object Identifier (DOI)
http://dx.doi.org/10.1172/JCI73906
Repository Citation
Davis, Frank; Rateri, Debra L.; and Daugherty, Alan, "Aortic Aneurysms in Loeys-Dietz Syndrome - A Tale of Two Pathways?" (2014). Saha Cardiovascular Research Center Faculty Publications. 7.
https://uknowledge.uky.edu/cvrc_facpub/7
Notes/Citation Information
Published in The Journal of Clinical Investigation, v. 124, issue 1, p. 79-81.
Copyright © 2014, American Society for Clinical Investigation
The copyright holder has granted permission for posting the article here.