The Role of Cullin-RING Ligases in Striated Muscle Development, Function, and Disease.

The Role of Cullin-RING Ligases in Striated Muscle Development, Function, and Disease.
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DOI:
10.3390/ijms21217936
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发表时间:
2020-10-26
影响因子:
5.6
通讯作者:
Lange S
Lange S
中科院分区:
生物学2区
文献类型:
--
作者:
Blondelle J;Biju A;Lange S

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横纹肌中蛋白质的精心策划的周转是肌细胞功能和存活所需的基本过程之一。复杂的蛋白质降解机制的功能障碍通常与心肌和骨骼肌肌病的发展有关。大多数肌肉蛋白质被泛素-蛋白酶体系统(UPS)降解。UPS涉及许多酶,包括E3-连接酶,其严格控制哪些蛋白质底物被蛋白酶体标记为降解。最近的数据显示,E3-连接酶的cullin家庭发挥更多样化和至关重要的作用,横纹肌比以前预期的。本文综述了cullin-RING E3-连接酶的多方面功能,其底物适配器,肌肉蛋白底物和调节蛋白,如Cop 9信号体,横纹肌的发展,以及它们在肌病病因学中的作用。
The well-orchestrated turnover of proteins in cross-striated muscles is one of the fundamental processes required for muscle cell function and survival. Dysfunction of the intricate protein degradation machinery is often associated with development of cardiac and skeletal muscle myopathies. Most muscle proteins are degraded by the ubiquitin–proteasome system (UPS). The UPS involves a number of enzymes, including E3-ligases, which tightly control which protein substrates are marked for degradation by the proteasome. Recent data reveal that E3-ligases of the cullin family play more diverse and crucial roles in cross striated muscles than previously anticipated. This review highlights some of the findings on the multifaceted functions of cullin-RING E3-ligases, their substrate adapters, muscle protein substrates, and regulatory proteins, such as the Cop9 signalosome, for the development of cross striated muscles, and their roles in the etiology of myopathies.
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