TGF-β–dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome

Abstract excerpt

Mitral valve prolapse (MVP) is a common human phenotype, yet little is known about the pathogenesis of this condition. MVP can occur in the context of genetic syndromes, including Marfan syndrome (MFS), an autosomal-dominant connective tissue disorder caused by mutations in fibrillin-1. Fibrillin-1 contributes to the regulated activation of the cytokine TGF-β, and enhanced signaling is a consequence of fibrillin-1...

Topics

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• Aortic Disease and Treatment Approaches
• Cardiac Valve Diseases and Treatments
• Connective tissue disorders research

Identifiers and source

Literature Corpus work

bbf8b443-e16e-5179-892b-2dac18a529ed

DOI

10.1172/jci200422715