Azienda Ospedaliera Universitaria Careggi and University of Florence, Florence, Italy.
Department of Internal Medicine, Section of Cardiovascular Medicine, Yale University, New Haven, CT, USA.
Eur J Intern Med. 2022 Aug;102:1-7. doi: 10.1016/j.ejim.2022.04.020. Epub 2022 May 6.
Over the past decade, the constant progress in science and technologies has provided innovative drug molecules that address specific disease mechanisms thus opening the era of drugs targeting the underlying pathophysiology of the disease. In this scenario, a new paradigm of modulation has emerged, following the development of small molecules capable of interfering with sarcomere contractile proteins. Potential applications include heart muscle disease and various forms of heart failure, although promising targets also include conditions affecting the skeletal muscle, such as degenerative neuromuscular diseases. In cardiac patients, a cardiac myosin stimulator, omecamtiv mecarbil, has shown efficacy in heart failure with reduced systolic function, lowering heart failure related events or cardiovascular death, while two inhibitors, mavacamten and aficamten, in randomized trials targeting hypertrophic cardiomyopathy, have been shown to reduce hypercontractility and left ventricular outflow obstruction improving functional capacity. Based on years of intensive basic and translational research, these agents are the prototypes of active pipelines promising to deliver an array of molecules in the near future. We here review the available evidence and future perspectives of myosin modulation in cardiovascular medicine.
在过去的十年中,科学技术的不断进步提供了创新的药物分子,针对特定的疾病机制,从而开启了针对疾病潜在病理生理学的药物治疗时代。在此背景下,一种新的调节模式出现了,这是随着能够干扰肌节收缩蛋白的小分子的发展而出现的。潜在的应用包括心肌疾病和各种形式的心力衰竭,尽管有前景的靶点还包括影响骨骼肌的疾病,如退行性神经肌肉疾病。在心脏患者中,肌球蛋白刺激剂奥马卡汀美卡比已显示出在收缩功能降低的心力衰竭中的疗效,降低心力衰竭相关事件或心血管死亡的风险,而两种抑制剂,马瓦卡坦和阿非卡坦,在针对肥厚型心肌病的随机试验中,已被证明可降低心肌过度收缩和左心室流出道梗阻,改善功能能力。基于多年的基础和转化研究,这些药物是活性药物的原型,有望在不久的将来提供一系列的药物。我们在此回顾了肌球蛋白调节在心血管医学中的现有证据和未来展望。