Lee Arnold
Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.
Drugs. 2025 Jun;85(6):833-837. doi: 10.1007/s40265-025-02159-z. Epub 2025 Apr 4.
Disassociation of transthyretin (TTR) has been identified as a key step in the pathology of TTR amyloid cardiomyopathy, which is characterised by the presence of amyloid fibrils in cardiac tissue. Acoramidis (ATTRUBY™) is a small molecule TTR stabiliser being developed by BridgeBio Pharma, Inc. and is the first drug to demonstrate near-complete (> 90%) stabilisation of TTR. This article summarizes the milestones in the development of acoramidis leading to its first approval in the USA for the treatment of the cardiomyopathy of wild-type or variant TTR-mediated amyloidosis in adults to reduce cardiovascular death and cardiovascular-related hospitalization. In the EU, a positive opinion has been adopted for the treatment of wild-type or variant transthyretin amyloidosis in adult patients with cardiomyopathy.
甲状腺素运载蛋白(TTR)的解离已被确定为TTR淀粉样心肌病病理过程中的关键步骤,其特征是心脏组织中存在淀粉样纤维。阿考米迪斯(ATTRUBY™)是BridgeBio Pharma公司正在研发的一种小分子TTR稳定剂,是首个证明能使TTR接近完全(>90%)稳定的药物。本文总结了阿考米迪斯的研发历程,该药物在美国首次获批用于治疗成人野生型或变异型TTR介导的淀粉样变性心肌病,以降低心血管死亡和心血管相关住院率。在欧盟,已对治疗成年心肌病患者的野生型或变异型甲状腺素运载蛋白淀粉样变性给予了积极意见。