Aortic aneurysms in loeys-dietz syndrome ?A tale of two pathways?

Frank Davis, Debra L. Rateri, Alan Daugherty

Research output: Contribution to journalReview articlepeer-review

9 Scopus citations

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-receptors. These mice exhibited several LDS-associated phenotypes. Despite being functionally defective, the mutated receptors enhanced TGF-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.

Original languageEnglish
Pages (from-to)79-81
Number of pages3
JournalJournal of Clinical Investigation
Volume124
Issue number1
DOIs
StatePublished - Jan 2 2014

ASJC Scopus subject areas

  • Medicine (all)

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