2017 Stress Proteins in Growth, Development and Disease GRC/GRS: Maintaining proteostasis over a lifetime.

项目摘要 细胞蛋白质组在一生中不断暴露于各种各样的蛋白毒性应激条件。 这些包括外部压力，如高温和辐射损伤，以及 在细胞增殖和分化过程中遇到的生理应激，例如氧化应激， 或遗传基因突变引起的蛋白质折叠的挑战。一种应激细胞的标志， 生物体是部署一个全面的应激反应必不可少的维持蛋白质 内稳态，包括分子伴侣的合成增加，有助于新生细胞的折叠， 多肽和防止蛋白质错误折叠和聚集。蛋白质质量控制和应激保护 机器需要严格的信号传导方式和转录程序，其中许多在转录过程中被改变。 许多疾病状态，包括癌症、心血管疾病、代谢疾病（例如，糖尿病）和 肝脏疾病应激反应的调节在寿命调节和衰老中也起着关键作用- 相关疾病如阿尔茨海默氏病、帕金森氏病、亨廷顿氏病以及朊病毒疾病。 2017年戈登研究会议“生长，发育和疾病中的应激蛋白质”将 强调了压力生物学和压力相关疾病的生物医学研究的最新进展。这 会议，在这个系列的第九次，将于7月9日至14日在星期日河度假村在纽里，缅因州。的 主席是Kevin Morano（UTHealth McGovern医学院，休斯顿），副主席是Elke Deuerling （康斯坦茨大学，德国）。特别强调将放在不同的模型系统， 用于研究压力传感，信号传导和基因表达调控，包括表观遗传 机制等在空间质量控制和蛋白质错误折叠管理方面的前沿工作 核糖体将被突出显示。间室蛋白质折叠状态和疾病之间的联系将是 探讨了会议的合议和学术环境鼓励积极讨论令人兴奋的 与压力研究的几个领域有关的发展。促进初级调查员的发展 在该领域，戈登研究研讨会将增加7月8日至9日，紧接在GRC之前。此外，本发明还 我们将继续最近的会议创新，如海报预告讲座，职业圆桌讨论， 方案拟订工作侧重于实地的妇女调查员。正式的科学计划，有限的出席率 有组织但非正式的互动机会使这次会议成为一个杰出的会议， 更深入地了解应激蛋白在人类健康、衰老和疾病中的多方面作用。

PROJECT SUMMARY The cellular proteome is constantly exposed to a wide variety of proteotoxic stress conditions over a lifetime. These include external stresses, such as elevated temperatures and radiation damage as well as physiological stresses encountered during cellular proliferation and differentiation, such as oxidative stress, or challenges to protein folding caused by inherited gene mutations. A hallmark of stressed cells and organisms is the deployment of a comprehensive stress response essential for the maintenance of protein homeostasis that includes increased synthesis of molecular chaperones that aid in the folding of nascent polypeptides and prevent protein misfolding and aggregation. The protein quality control and stress protection machineries require strict signaling modalities and transcriptional programs, many of which are altered in a number of disease states, including cancer, cardiovascular disease, metabolic disease (e.g., diabetes) and liver disease. Modulation of the stress response also plays a critical role in life-span regulation and aging- related disorders such as Alzheimer's, Parkinson's, Huntington's diseases as well as prion-based disease. The 2017 Gordon Research Conference on "Stress Proteins in Growth, Development and Disease" will highlight the most recent advances in biomedical research of stress biology and stress-related diseases. This conference, the ninth in this series, will be held July 9-14 at the Sunday River Resort in Newry, Maine. The chair is Kevin Morano (UTHealth McGovern Medical School, Houston) and the vice-chair is Elke Deuerling (University of Konstanz, Germany). Special emphasis will be placed on diverse model systems that are being used to investigate stress sensing, signaling and regulation of gene expression, including epigenetic mechanisms. Cutting-edge work on spatial quality control and management of protein misfolding at the ribosome will be highlighted. Connections between compartmental protein folding status and disease will be explored. The conference's collegial and scholarly environment encourages vigorous discussions of exciting developments related to several areas of stress research. To promote the development of junior investigators in the field, a Gordon Research Seminar will be added July 8-9, immediately preceding the GRC. Additionally, we will continue recent meeting innovations of poster teaser talks, career roundtable discussions, programming focused on women investigators in the field. The formal scientific program, limited attendance and organized but informal opportunities for interaction make this meeting a preeminent conference promoting deeper understanding of the versatile roles of stress proteins in human health, aging and disease.