Quality Control System of the Endoplasmic Reticulum and Related Diseases  

作　　者：Jun-Chao WU Zhong-Qin LIANG Zheng-Hong QIN 

机构地区：[1]Department of Pharmacology, School of Medicine, Soochow University, Suzhou 215123, China

出　　处：《Acta Biochimica et Biophysica Sinica》2006年第4期219-226,共8页生物化学与生物物理学报（英文版）

基　　金：This work was supported by the grants from the National Natural Science Foundation of China (No.30370506);the Doctoral Program of Higher Education of China (No.20050285017)

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.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.

关 键 词：protein folding quality control calnexin/calreticulin cycle endoplasmic reticulum-associated degradation protein misfolding disease 

分 类 号：Q2[生物学—细胞生物学]