MITGEST: Quality Control of the Mitochondrial Gene Expression System in Health and Disease

线粒体产生人体通过食物获得的大部分能量，因此在细胞代谢中起着关键作用。线粒体生物学的核心是细胞器中的DNA (mtDNA)：它的维护、正确的RNA加工和有效的翻译都是必不可少的，必须协调以确保线粒体的正常功能和细胞生理。mtDNA的维持或表达缺陷会改变能量和营养代谢，这越来越被认为会影响医学学科，从数百种遗传疾病到神经退行性疾病、免疫学、传染病、癌症和衰老。迫切需要深入了解线粒体基因表达系统和新方法，以开发以mtDNA扰动为特征的全套线粒体疾病的治疗方法。MITGEST将把mtDNA维持和表达方面的世界级学术卓越与私营部门在核酸化学和线粒体疗法开发方面的专业知识结合起来，以解决这一迫切需求。作为该领域第一个采用完全整合方法的研究培训计划，我们的10名博士候选人（DC）将阐明mtDNA是如何维持和表达的，并支持开发分析线粒体中RNA和DNA代谢的新方法。这些进展将补充和促进/加强旨在减轻一系列人类疾病线粒体功能障碍的创新治疗策略和实验医学研究。我们的8个受益人和来自8个欧洲国家和以色列的11个相关合作伙伴网络建立在成熟的合作基础上，并建立了新的伙伴关系，为我们的10名博士生提供跨部门和多学科的科学和可转移技能培训，以培养下一代能够利用线粒体生物学和医学最新进展的高技能科学家。

Mitochondria produce most of the energy the body acquires through food, and are thus pivotal players in cellular metabolism.Central to mitochondrial biology is the DNA in the organelle (mtDNA): its maintenance, correct RNA processing, and efficient translation are all essential and must be coordinated to ensure proper mitochondrial function and cellular physiology. Defective maintenance or expression of mtDNA alters energy and nutrient metabolism, which are increasingly recognized to impact medical disciplines, ranging from hundreds of genetic disorders to neurodegeneration, immunology, transmissible disease, cancer and ageing. A deep understanding of the mitochondrial gene expression system and new methods are urgently needed to develop treatments for the full panoply of mitochondrial disorders that feature mtDNA perturbation.MITGEST will unite world-class academic excellence in mtDNA maintenance and expression with private sector expertise in nucleic acid chemistry and the development of mitochondrial therapeutics to address this urgent need. As the first research training programme in this field to take a fully integrated approach, our 10 doctoral candidates (DC) will elucidate how mtDNA is maintained and expressed and support the development of new methods to analyse RNA and DNA metabolism in mitochondria. These advances will complement and facilitate/enhance innovative treatment strategies and experimental medicine studies designed to mitigate mitochondrial dysfunction in a range of human diseases.Our network of 8 beneficiaries and 11 associated partners from 8 European countries and Israel builds on proven collaborations and establishes new partnerships to deliver intersectoral and multidisciplinary scientific and transferable skills training to our 10 PhD students to generate the next generation of highly-skilled scientists able to exploit the latest advances in mitochondrial biology and medicine.