Mechanism of proteotoxic stress induced type I interferon signalling and implications for human disease

蛋白毒性应激诱导 I 型干扰素信号传导的机制及其对人类疾病的影响

• 批准号: nhmrc : 1143412
• 金额: $ 21.82万
• 依托单位: The Walter and Eliza Hall Institute of Medical Research
• 依托单位国家: 澳大利亚
• 项目类别: Early Career Fellowships
• 财政年份: 2018
• 资助国家: 澳大利亚
• 起止时间: 2018-01-01 至 2021-12-31
• 项目状态: 已结题
• 来源: https://researchdata.edu.au/mechanism-proteotoxic-stress-human-disease/1319953
• 关键词: Mechanism; proteotoxic; stress; induced; type

All cells have a proteasome system to degrade unwanted proteins. Proteasome dysfunction causes a build-up of proteins that triggers, through an unknown mechanism, activation of the immune system leading to inflammation. People with mutations in genes which code for proteasome activity experience a severe disease known as Proteasome-Associated Autoinflammatory Syndrome. We aim to elucidate the link between protein aggregation and immune activation and employ this knowledge in disease treatment.

所有细胞都有一个蛋白酶体系统来降解不需要的蛋白质。蛋白酶体功能障碍导致蛋白质的积聚，通过一种未知的机制触发免疫系统的激活，导致炎症。编码蛋白酶体活性的基因突变的人会经历一种被称为蛋白酶体相关自炎综合症的严重疾病。我们的目标是阐明蛋白质聚集和免疫激活之间的联系，并将这一知识用于疾病治疗。