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Aortic aneurysms in Loeys-Dietz syndrome - a tale of two pathways?

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JOURNAL OF CLINICAL INVESTIGATION
卷 124, 期 1, 页码 79-81

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AMER SOC CLINICAL INVESTIGATION INC
DOI: 10.1172/JCI73906

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  1. NHLBI NIH HHS [R01 HL062846, R01 HL107319, HL107319, HL062846] Funding Source: Medline

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

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