...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>International Journal of Molecular Sciences >The Ubiquitin Proteasome System in Neuromuscular Disorders: Moving Beyond Movement
【24h】

The Ubiquitin Proteasome System in Neuromuscular Disorders: Moving Beyond Movement

机译：神经肌肉障碍中泛素蛋白酶体系：超越运动

获取原文
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Neuromuscular disorders (NMDs) affect 1 in 3000 people worldwide. There are more than 150 different types of NMDs, where the common feature is the loss of muscle strength. These disorders are classified according to their neuroanatomical location, as motor neuron diseases, peripheral nerve diseases, neuromuscular junction diseases, and muscle diseases. Over the years, numerous studies have pointed to protein homeostasis as a crucial factor in the development of these fatal diseases. The ubiquitin–proteasome system (UPS) plays a fundamental role in maintaining protein homeostasis, being involved in protein degradation, among other cellular functions. Through a cascade of enzymatic reactions, proteins are ubiquitinated, tagged, and translocated to the proteasome to be degraded. Within the ubiquitin system, we can find three main groups of enzymes: E1 (ubiquitin-activating enzymes), E2 (ubiquitin-conjugating enzymes), and E3 (ubiquitin–protein ligases). Only the ubiquitinated proteins with specific chain linkages (such as K48) will be degraded by the UPS. In this review, we describe the relevance of this system in NMDs, summarizing the UPS proteins that have been involved in pathological conditions and neuromuscular disorders, such as Spinal Muscular Atrophy (SMA), Charcot–Marie–Tooth disease (CMT), or Duchenne Muscular Dystrophy (DMD), among others. A better knowledge of the processes involved in the maintenance of proteostasis may pave the way for future progress in neuromuscular disorder studies and treatments.
机译：神经肌肉障碍（NMDS）在全球3000人中影响1。有超过150种不同类型的NMD，其中共同的特征是肌肉力量的损失。这些疾病根据其神经杀菌位置进行分类，作为运动神经元疾病，周围神经疾病，神经肌肉结疾病和肌肉疾病。多年来，许多研究指出蛋白质稳态作为这些致命疾病发展的关键因素。泛素 - 蛋白酶体系统（UPS）在维持蛋白质稳态中起着基本作用，参与蛋白质降解，以及其他细胞功能。通过酶促反应级联，蛋白质遍布，标记，并向蛋白酶转化为降解。在泛素系统内，我们可以找到三个主要的酶组：E1（泛素 - 活化酶），E2（遍兴蛋白 - 缀合酶）和E3（泛素 - 蛋白质连接酶）。只有具有特定链键（例如K48）的泛染蛋白将被UPS降解。在本次审查中，我们描述了该系统在NMDS中的相关性，总结了已涉及病理病症和神经肌肉疾病的UPS蛋白，例如脊柱肌肉萎缩（SMA），Charcot-Marie-Tooth疾病（CMT）或Duchenne肌营养不良（DMD）等。更好地了解涉及蛋白质抑制的过程的过程可能会为未来神经肌病研究和治疗中的未来进展铺平道路。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号