Department of Physiology, Pennsylvania Muscle Institute, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
Nat Med. 2018 Aug;24(8):1225-1233. doi: 10.1038/s41591-018-0046-2. Epub 2018 Jun 11.
Detyrosinated microtubules provide mechanical resistance that can impede the motion of contracting cardiomyocytes. However, the functional effects of microtubule detyrosination in heart failure or in human hearts have not previously been studied. Here, we utilize mass spectrometry and single-myocyte mechanical assays to characterize changes to the cardiomyocyte cytoskeleton and their functional consequences in human heart failure. Proteomic analysis of left ventricle tissue reveals a consistent upregulation and stabilization of intermediate filaments and microtubules in failing human hearts. As revealed by super-resolution imaging, failing cardiomyocytes are characterized by a dense, heavily detyrosinated microtubule network, which is associated with increased myocyte stiffness and impaired contractility. Pharmacological suppression of detyrosinated microtubules lowers the viscoelasticity of failing myocytes and restores 40-50% of lost contractile function; reduction of microtubule detyrosination using a genetic approach also softens cardiomyocytes and improves contractile kinetics. Together, these data demonstrate that a modified cytoskeletal network impedes contractile function in cardiomyocytes from failing human hearts and that targeting detyrosinated microtubules could represent a new inotropic strategy for improving cardiac function.
去酪氨酸化微管提供了机械阻力,可阻碍收缩性心肌细胞的运动。然而,微管去酪氨酸化在心力衰竭或人类心脏中的功能影响以前尚未被研究过。在这里,我们利用质谱和单细胞力学测定来描述心力衰竭患者中心肌细胞细胞骨架的变化及其功能后果。左心室组织的蛋白质组学分析揭示了衰竭的人心肌中中间丝和微管的一致上调和稳定。正如超分辨率成像所揭示的那样,衰竭的心肌细胞的特征是密集的、高度去酪氨酸化的微管网络,这与肌细胞硬度增加和收缩功能受损有关。用药物抑制去酪氨酸化微管可降低衰竭心肌细胞的粘弹性,并恢复 40-50%丧失的收缩功能;用遗传方法减少微管去酪氨酸化也可使心肌细胞变软并改善收缩动力学。总之,这些数据表明,改变的细胞骨架网络会阻碍衰竭人心肌细胞的收缩功能,靶向去酪氨酸化微管可能代表改善心脏功能的一种新的变力策略。