2019 Stress Proteins in Growth, Development, and Disease GRC/GRS

项目摘要 细胞、组织和生物体的生存能力关键取决于维持蛋白质稳态的能力 因此，它们不断地使细胞蛋白质组适应外部和内部的变化。 这些变化包括环境条件的变化、不同的发育阶段、急性应激 例如高温、炎症和氧化应激，或由突变或 衰老所有这些事件都构成了引起细胞内稳态丧失的风险因素， 蛋白质错误折叠和聚集，最终导致细胞死亡。这种蛋白质抑制的下降尤其是 在衰老过程中以及对于许多疾病，包括神经变性，癌症，心血管， 或代谢紊乱（例如，糖尿病）。因此，细胞感知和响应变化的能力 条件和压力对正常细胞生长和发育至关重要，并有助于防止衰老- 相关疾病。越来越多的证据表明，细胞激活复杂的应激反应， 适应不断变化的条件，包括特定的信号机制，复杂的转录和 翻译重编程和细胞质量控制系统的激活，如蛋白水解 防止有毒错误折叠和聚集蛋白积聚的途径和分子伴侣 已知在寿命调节和老年性疾病（如阿尔茨海默氏症）中起关键作用， 帕金森氏症、亨廷顿氏症以及朊病毒疾病。 2019年戈登研究会议“生长，发育和疾病中的应激蛋白”在 2019年6月23日至28日，在意大利的文艺复兴时期的托斯卡纳Il Ciocco是这个成功系列的第十个， 将突出细胞应激生物学领域的最新前沿进展，从基础 压力程序和蛋白质质量控制系统的基本机制，直至干预策略。 特别强调将放在新的方面，关于压力信号和基因调控 表达，相分离和其他特定的沉积物的RNA和蛋白质的压力，消除 有毒蛋白质种类的策略，以及细胞器应激反应对病理的作用。另外我们 将继续最近成功的会议创新：戈登研究研讨会（GRS）将增加 从6月22日至23日，紧接在GRC之前，以促进早期职业科学家的发展 (PhD学生和博士后）在该领域。海报预览、职业圆桌讨论和“权力 以妇女调查员为重点的“一小时”将结束这次会议。正式的科学计划，有限的 出席率，以及有组织但非正式的互动机会，使这次会议成为一个卓越的 会议在科学的前沿，促进更深入地了解压力蛋白质的影响， 人类健康、老龄化和疾病的有效对策和发展。椅子是埃尔克 Deuerling（德国康斯坦茨大学），副主席是Brain C。Freeman（美国伊利诺伊大学）。

PROJECT SUMMARY The viability of cells, tissues and organisms critically depend on the ability to maintain protein homeostasis (proteostasis) and thus, they constantly adapt the cellular proteome to external and internal alterations. Such alterations include changing environmental conditions, different developmental stages, acute stress such as elevated temperatures, inflammation and oxidative stress, or chronic stress caused by mutations or aging. All these incidents constitute risk factors that provoke a loss of cellular homeostasis that can lead to protein misfolding and aggregation and, eventually, cell death. Such a decline in proteostasis is especially prominent during aging and for a number of diseases including neurodegeneration, cancer, cardiovascular, or metabolic disorders (e.g., diabetes). Thus, the ability of cells to sense and respond to changing conditions and stress is critical for normal cell growth and development, and helps to protect against aging- related diseases. A growing body of evidence shows that cells activate elaborated stress responses to adapt to changing conditions including specific signaling regimes, a complex transcriptional and translational reprogramming, and the activation of cellular quality control systems such as proteolytic pathways and molecular chaperones to prevent accumulation of toxic misfolded and aggregated proteins known to play 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 2019 Gordon Research Conference on "Stress Proteins in Growth, Development and Disease" held in June 23-28, 2019, at the Renaissance Tuscany Il Ciocco in Italy is the tenth in this successful series and will highlight most recent cutting-edge advances in the field of cell stress biology, ranging from underlying basic mechanisms of stress programs and of protein quality control systems up to intervention strategies. Special emphasis will be placed on novel aspects regarding stress signaling and regulation of gene expression, phase separation and other specific deposits of RNA and proteins under stress, elimination strategies of toxic protein species, and the role of organelle stress responses to pathology. Additionally, we will continue recent successful meeting innovations: the Gordon Research Seminar (GRS) will be added from June 22-23, immediately preceding the GRC, to promote the development of early career scientists (PhD students and post-docs) in the field. Poster previews, career roundtable discussions and a “power hour” focused on women investigators will complete this conference. The formal scientific program, limited attendance, and organized but informal opportunities for interaction make this meeting a pre-eminent conference at the forefront of science promoting a deeper understanding of the impact of stress proteins in human health, aging and disease and the development of efficient countermeasures. The chair is Elke Deuerling (Univ. of Konstanz, Germany) and the vice-chair is Brain C. Freeman (Univ. of Illinois, USA).