Article
Angiotensin II-dependent TGF-β signaling contributes to Loeys-Dietz syndrome vascular pathogenesis.
The Journal of clinical investigation - 1 Jan 2014
Gallo Elena M, Loch David C, Habashi Jennifer P, Calderon Juan F, Chen Yichun, Bedja Djahida, van Erp Christel, Gerber Elizabeth E, Parker Sarah J, Sauls Kimberly, Judge Daniel P, Cooke Sara K, Lindsay Mark E, Rouf Rosanne, Myers Loretha, ap Rhys Colette M, Kent Kathleen C, Norris Russell A, Huso David L, Dietz Harry C
Abstract excerpt
Loeys-Dietz syndrome (LDS) is a connective tissue disorder that is characterized by a high risk for aneurysm and dissection throughout the arterial tree and phenotypically resembles Marfan syndrome. LDS is caused by heterozygous missense mutations in either TGF-β receptor gene (TGFBR1 or TGFBR2), which are predicted to result in diminished TGF-β signaling; however, aortic surgical samples from patients show...
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