中文摘要：

endoplasmic 蜂窝胃的质量控制(质量管理) 系统(嗯) 是在蛋白质成熟过程的重要监视机制，它从 ER 保证适当地合拢的蛋白质的出口。不正确地或不完全地合拢的蛋白质被嗯住 proteasome 为 refolding 或降级在 ER 保留。calnexin/calreticulin 周期和嗯联系降级是在质量管理的关键元素。这二机制一起工作允许不正确地合拢的蛋白质有另外的机会完成他们的本国的符合构造。质量管理机能障碍涉及变异的蛋白质引起的许多疾病，许多哪个是 neurodegenerative 混乱的原因。在 QC 系统的分子的规定的更好的理解将揭开蛋白质错误褶层和帮助引起的许多疾病的分子的病原的机制为阻止或对待这些疾病发现新奇策略。

英文摘要：

The quality control （QC） system of the endoplasmic reticulum （ER） is an important monitoring mechanism in the protein maturation process, which ensures export of properly folded proteins from the ER. Incorrectly or incompletely folded proteins are retained in the ER for refolding or degradation by the ER-residing proteasome. The calnexin/calreticulin cycle and ER-associated degradation are the key elements in QC. These two mechanisms work together to allow incorrectly folded proteins have additional opportunities to achieve their native conformations. The QC dysfunction is involved in many diseases caused by mutant proteins, many of which are causes of neurodegenerative disorders. A better understanding of molecular regulation in the QC system will uncover the molecular pathogenic mechanisms of many diseases caused by protein misfolding and help discover novel strategies for preventing or treating these diseases.