Aridor Meir
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, 3500 Terrace St. Pittsburgh PA 15261, USA. aridor+@pitt.edu
Adv Drug Deliv Rev. 2007 Aug 10;59(8):759-81. doi: 10.1016/j.addr.2007.06.002. Epub 2007 Jun 21.
The endoplasmic reticulum (ER) is a central processor that controls the expression of functional proteins, required for the communication of the cell with the external environment. Plasma membranes receptors, ion channels, secreted hormones, catabolic and metabolic enzymes are folded and assembled in the ER. Key metabolic functions are also regulated from the ER. Molecular quality control monitors ER processing activities and co-ordinates these activities with cell and organism demands. Recent understandings of the molecular basis for ER processing activities illuminate the key role of the ER in the development of a variety of diseases. ER derived diseases include specific genetic disorders such as cystic fibrosis or highly prevalent diseases including diabetes and a range of neurodegenerative diseases. ER processing also plays a key role in the development of cancer. This review summarizes the molecular basis for ER processing functions and current avenues in ER-targeted drug development.
内质网（ER）是一个核心处理器，它控制着功能性蛋白质的表达，而这些蛋白质是细胞与外部环境进行通讯所必需的。质膜受体、离子通道、分泌激素、分解代谢和代谢酶在内质网中折叠和组装。关键的代谢功能也由内质网调节。分子质量控制监测内质网的加工活动，并根据细胞和机体的需求协调这些活动。最近对内质网加工活动分子基础的认识阐明了内质网在多种疾病发生发展中的关键作用。内质网相关疾病包括特定的遗传疾病，如囊性纤维化，或高发性疾病，包括糖尿病和一系列神经退行性疾病。内质网加工在癌症的发生发展中也起着关键作用。本综述总结了内质网加工功能的分子基础以及目前内质网靶向药物开发的途径。
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