Under construction: The dynamic assembly, maintenance, and degradation of the cardiac sarcomere.
Under construction: The dynamic assembly, maintenance, and degradation of the cardiac sarcomere.
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DOI：
10.1016/j.yjmcc.2020.08.018
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发表时间：
2020-11
影响因子：
5
通讯作者：
Kirk JA
中科院分区：
文献类型：
--
作者：
Martin TG;Kirk JA
肌节是横纹肌的基本收缩单位，是一种高度有序的蛋白质复合物，其核心是肌动蛋白和肌球蛋白丝。在发育过程中将肌节成分组装成这种有组织的结构，并随着新肌节的建立而生长，这是一个由许多因素协调的复杂过程。一旦组装，肌节需要不断的维护，因为它的持续收缩伴随着升高的机械，热和氧化应激，这使蛋白质易于错误折叠和毒性聚集。为了防止蛋白质错误折叠并保持肌节完整性，肌节通过各种蛋白质质量控制（PQC）机制进行监测。心肌细胞对有效PQC的需求增加，因为这些细胞是终末分化的，必须存活多年，同时保持最佳的机械输出。为了防止毒性蛋白质聚集，分子伴侣稳定变性的肌节蛋白并促进其重折叠。然而，当旧的和错误折叠的蛋白质不能被分子伴侣挽救时，它们必须通过降解途径回收：钙蛋白酶和泛素-蛋白酶体系统，它们在基础条件下工作，以及应激反应性自噬-溶酶体途径。突变和缺乏分子伴侣和相关的因素负责肌节的维护通常会导致肌节结构紊乱和心脏病的进展，突出了有效的肌节PQC维持心脏功能的必要性。本文综述了肌节组装和营业额的动态调节，重点是参与这些过程的分子伴侣，并描述了分子伴侣的改变-通过突变和表达缺陷-与心力衰竭的疾病进展有关。
The sarcomere is the basic contractile unit of striated muscle and is a highly ordered protein complex with the actin and myosin filaments at its core. Assembling the sarcomere constituents into this organized structure in development, and with muscle growth as new sarcomeres are built, is a complex process coordinated by numerous factors. Once assembled, the sarcomere requires constant maintenance as its continuous contraction is accompanied by elevated mechanical, thermal, and oxidative stress, which predispose proteins to misfolding and toxic aggregation. To prevent protein misfolding and maintain sarcomere integrity, the sarcomere is monitored by an assortment of protein quality control (PQC) mechanisms. The need for effective PQC is heightened in cardiomyocytes as these cells are terminally differentiated and must survive for many years while preserving optimal mechanical output. To prevent toxic protein aggregation, molecular chaperones stabilize denatured sarcomere proteins and promote their refolding. However, when old and misfolded proteins cannot be salvaged by chaperones, they must be recycled via degradation pathways: the calpain and ubiquitin-proteasome systems, which operate under basal conditions, and the stress-responsive autophagy-lysosome pathway. Mutations to and deficiency of molecular chaperones and the associated factors charged with sarcomere maintenance commonly lead to sarcomere structural disarray and the progression of heart disease, highlighting the necessity of effective sarcomere PQC for maintaining cardiac function. This review focuses on the dynamic regulation of assembly and turnover at the sarcomere with an emphasis on the chaperones involved in these processes and describes the alterations to chaperones – through mutations and deficient expression – implicated in disease progression to heart failure.
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影响因子：
16.6
作者：
Boczkowska M;Rebowski G;Kremneva E;Lappalainen P;Dominguez R
通讯作者：
Dominguez R
DOI：
10.1073/pnas.96.11.6137
发表时间：
1999-05-25
影响因子：
11.1
作者：
Bova, MP;Yaron, O;Horwitz, J
通讯作者：
Horwitz, J
影响因子：
16
作者：
Andrianantoandro, Ernesto;Pollard, Thomas D.
通讯作者：
Pollard, Thomas D.
肌动蛋白去聚合因子（ADF/Cofilin）提高了细丝流动率：基于肌动蛋白的运动的影响。
DOI：
10.1083/jcb.136.6.1307
发表时间：
1997-03-24
期刊：
The Journal of cell biology
影响因子：
--
作者：
Carlier MF;Laurent V;Santolini J;Melki R;Didry D;Xia GX;Hong Y;Chua NH;Pantaloni D
通讯作者：
Pantaloni D
smyd1a 和 smyd1b 斑马鱼突变体中肌节组装缺陷
影响因子：
4.8
作者：
Cai, Mengxin;Han, Lichen;Du, Shaojun
通讯作者：
Du, Shaojun