title: Aortic aneurysms in Loeys-Dietz syndrome - a tale of two pathways?
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.
authors.forename: Frank
authors.initials: F
authors.lastname: Davis
authors.forename: Debra L
authors.initials: DL
authors.lastname: Rateri
authors.forename: Alan
authors.initials: A
authors.lastname: Daugherty
mesh_terms: D000804:Angiotensin II / Q000502:physiology*; D000818:Animals; D001014:Aortic Aneurysm / Q000378:metabolism*; D005260:Female; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000378:metabolism*; D016212:Transforming Growth Factor beta / Q000378:metabolism*
chemical_list: D016212:Transforming Growth Factor beta; D000804:Angiotensin II