Cell biology. Heart brakes.
Cell biology. Heart brakes.
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细胞生物学。
DOI：
10.1126/science.1227943
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发表时间：
2012
期刊：
影响因子：
--
通讯作者：
Ajtai,Katalin
中科院分区：
文献类型：
--
作者：
Burghardt,ThomasP;Ajtai,Katalin
我们中的许多人都敏锐地意识到肌肉疾病，因为它们经常影响我们的生活。例如，心力衰竭是导致死亡的主要原因，它的遗传联系将研究重点放在了对肌肉蛋白编码基因的测序上。几种肌肉蛋白与心脏病有关，最显著的是丰富的肌球蛋白和相对缺乏的结构蛋白肌球蛋白结合蛋白C(MyBP-C)。肌球蛋白产生心肌收缩的力量，但心脏在没有MyBP-C的情况下会跳动，它在收缩和疾病中的作用尚不清楚。此外，MyBP-C的突变会导致较晚的心力衰竭，掩盖了蛋白质功能障碍与疾病机制之间的联系。这些挑战挑战了自下而上的方法来描述孤立的MyBP-C的体外功能，但是心脏太复杂了，不能自上而下地描述整个肌肉的特征。Previset等人在本期第1215页进行的新研究采取了中间路线，使用了一种简洁的实验系统，该系统保留了相关的组织结构，同时允许观察单粒子动力学，以精确定位MyBP-C在肌肉收缩中的作用。
Many of us are keenly aware of muscle diseases because they frequently impact our lives. Heart failure, for example, is a leading cause of death, and its hereditary link has focused research efforts on sequencing the genes encoding muscle proteins . Several muscle proteins are implicated in heart disease, most notably the abundant motor protein myosin, and the relatively scarce structural protein myosin binding protein C (MyBP-C). Myosin generates the force for heart muscle contraction, but a heart will pump without MyBP-C, and its role in contraction and disease was unknown. In addition, mutations in MyBP-C cause late onset of heart failure, obscuring the link between protein malfunction and disease mechanism . These challenges have defied a bottom-up approach to characterize isolated MyBP-C's function in vitro, but the heart is too complex for top-down whole-muscle characterization . New research by Previset al.on page 1215 in this issue takes the middle road, using a concise experimental system that preserves relevant tissue structure while allowing the observation of single-particle dynamics to pinpoint MyBP-C's role in muscle contraction.