The Antioxidant/Nitric Oxide-Quenching Agent Cobinamide Prevents Aortic Disease in a Mouse Model of Marfan Syndrome
JACC: Basic to Translational Science(2024)
摘要
•MFS is a frequent cause of familial thoracic aortic aneurysms, and mortality from aortic rupture remains high, despite current treatments with β-adrenergic and angiotensin receptor blockers.•Based on recent evidence implicating oxidative stress and excess NO signaling in the aortic pathology of MFS, we studied the novel antioxidant and NO-quenching agent cobinamide in a MFS mouse model.•Cobinamide normalized markers of oxidative and nitrosative stress, prevented characteristic pathologic changes in the aorta, and reduced aortic dilation.•Cobinamide had favorable safety and pharmacokinetic profiles, suggesting it could be a novel disease-modifying treatment for MFS.
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关键词
aortic aneurysm,Fbn1C1041G/+ mutant mice,nitrosative stress,oxidative stress,protein kinase G
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