转甲状腺素
四聚体
医学
心肌病
离解(化学)
淀粉样疾病
淀粉样蛋白(真菌学)
淀粉样纤维
内科学
生物化学
心力衰竭
病理
淀粉样β
化学
疾病
酶
物理化学
作者
Teresa Maria Capovilla,Andrea Lalario,Maddalena Rossi,Aldostefano Porcari,Alberto Aimo,Giuseppe Limongelli,Michele Emdin,Marco Merlo,Gianfranco Sinagra
标识
DOI:10.1016/j.hfc.2024.03.007
摘要
Transthyretin amyloid cardiomyopathy (ATTR-CM) is caused by the myocardial extracellular deposition of amyloid fibrils formed from the dissociation of TTR tetramer into monomers. The rate-limiting step in TTR amyloidogenesis is the dissociation of the TTR tetramer into monomers: Tafamidis is an effective TTR-stabilizer in its native homotetrameric structure. Tafamidis is a safe and effective drug in reducing symptoms, hospitalization and mortality in accurately selected patients affected by hereditary and wild-type transthyretin amyloid cardiomyopathy.
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