Sitia Roberto, Braakman Ineke
Università Vita-Salute San Raffaele Scientific Institute, 20132 Milano, Italy.
Nature. 2003 Dec 18;426(6968):891-4. doi: 10.1038/nature02262.
The endoplasmic reticulum (ER) is a factory where secretory proteins are manufactured, and where stringent quality-control systems ensure that only correctly folded proteins are sent to their final destinations. The changing needs of the ER factory are monitored by integrated signalling pathways that constantly adjust the levels of folding assistants. ER chaperones and signalling molecules are emerging as drug targets in amyloidoses and other protein-conformational diseases.
内质网（ER）是一个制造分泌蛋白的工厂，也是一个严格的质量控制系统确保只有正确折叠的蛋白质才能被送往其最终目的地的场所。内质网工厂不断变化的需求由整合的信号通路进行监测，这些信号通路会不断调整折叠辅助蛋白的水平。在内淀粉样变性和其他蛋白质构象疾病中，内质网伴侣蛋白和信号分子正逐渐成为药物靶点。
