2012 Iron Sulfur Enzymes Gordon Research Conference

2012年铁硫酶戈登研究会议

基本信息

批准号：
8390766
负责人：
Markus W Ribbe
金额：
$ 0.8万
依托单位：
GORDON RESEARCH CONFERENCES
依托单位国家：
美国
项目类别：
财政年份：
2012
资助国家：
美国
起止时间：
2012-08-01 至 2013-07-31
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/8390766
关键词：
Acids Address Animals Area Attributes of Chemicals Biogenesis Biological Process Biology Carbon Dioxide Catalysis Cells Chemistry DNA Development Diagnostic Disease Economics Electron Transport Enzymes Failure Friedreich Ataxia Gene Mutation Genetic Transcription Goals Health Heart failure Heme Iron Human Impairment Industry Investigation Iron Iron-Sulfur Proteins Knowledge Lactic Acidosis Lead Life Longevity Medical Medical Research Medicine Metabolism Molecular Structure Muscle function Myopathy Names National Institute of Diabetes and Digestive and Kidney Diseases Nitrogenase Organism Oxidation-Reduction Pathway interactions Patients Plants Process Proteins RNA Reaction Regulation Request for Applications Research Research Personnel Role Seizures Series Skeletal Muscle Societies Spectrum Analysis Sulfur Sulofenur Technology Time Translations Work base career catalyst chemical bond cofactor college effective therapy emerging adult health training human disease infancy insight meetings novel therapeutics progressive neurodegeneration symposium treatment strategy

项目摘要

DESCRIPTION (provided by applicant): This application requests partial support for a meeting on Iron Sulfur Enzymes as part of a the Gordon Research Conference series to be held in Mount Holyoke College (South Hadley, MA) on June 10-15, 2012 with a broad and long-term goal of bringing together the world's leading experts in iron-sulfur clusters, focusing on enzymatic mechanisms, biogenesis, roles in regulation and in human disease in a uniquely cross-disciplinary manner. First organized in 1994 to focus on nitrogenase and its unique iron sulfur chemistry, knowledge about iron sulfur clusters has grown exponentially in the last two decades and, in 2006, the name of the conference was changed accordingly to Gordon Conference on Iron Sulfur Enzymes. The conference has tremendous breadth, featuring sessions on numerous iron sulfur enzymes of unicellular organisms, animals and plants, which provide important insights into how these versatile proteins are synthesized and utilized. Proteins containing iron-sulfur cofactors perform a variety of biological functions, ranging across electron transfer, acid- base catalysis, and sensing where they are agents for cell regulation through transcription (DNA) or translation (RNA). They are redox catalysts for radical-based reactions and the activation of H2, N2 and CO2, processes that offer scientific and economic challenges for industry. Iron-sulfur centers provide the focus for fundamental investigations of chemical bonding, spectroscopy, structure and molecular mechanism, and their functions have numerous implications for health, medicine and applications for technology, including renewable energy. The specific aim of this conference is to include distinguished speakers and discussion leaders from many fields, and topics will range from mechanistic discussions of bacterial enzymes to discussions of an emerging class of human diseases characterized by abnormalities in their iron-sulfur cluster biogenesis pathway. The health relatedness of the meeting is clear and will be discussed extensively. Three new diseases that result from mutations of genes in the iron-sulfur biogenesis pathway have been discovered since 2010, and there are no effective therapies for any of the diseases, including Friedreich ataxia, a disease characterized by progressive neurodegeneration and progressive heart failure. Novel therapeutic strategies are needed, and mixing the chemists and medical doctors together for the first time has the potential to catalyze progress in the field. PUBLIC HEALTH RELEVANCE: Iron sulfur cofactors are essential for virtually all forms of life, and much progress has been made on understanding their special chemical attributes and their unique contributions to metabolism. In the last ten years, it has become clear that failure to correctly synthesize iron sulfur clusters and proteins causes a range of rare and serious diseases, ranging from infantile lactic acidosis and seizures to Friedreich ataxia, a disease in which patients survive until early adulthood, and ISCU myopathy, in which patients have a normal lifespan, but have marked impairment of skeletal muscle function. The discussion of current research at this Gordon Research Conference will define important questions and problems that are associated with a growing list of unique diseases, and this is a crucial opportunity to bring researchers together to work on development of mechanistic insight into how iron-sulfur clusters are assembled and transferred, which may lead to new treatment strategies for diseases of iron sulfur cluster deficiency.

描述（由申请人提供）：本申请请求对铁硫酶会议提供部分支持，该会议是戈登研究会议系列的一部分，将于 2012 年 6 月 10 日至 15 日在曼荷莲学院（马萨诸塞州南哈德利）举行广泛而长期的目标是汇集世界领先的铁硫簇专家，以独特的跨学科方式重点研究酶促机制、生物发生、调节和人类疾病中的作用。该会议于 1994 年首次组织，专注于固氮酶及其独特的铁硫化学，在过去二十年中，有关铁硫簇的知识呈指数级增长，2006 年，会议名称相应更改为戈登铁硫酶会议。该会议内容广泛，以单细胞生物、动物和植物的众多铁硫酶为特色，为如何合成和利用这些多功能蛋白质提供了重要见解。含有铁硫辅助因子的蛋白质具有多种生物学功能，包括电子转移、酸碱催化和传感，它们是通过转录 (DNA) 或翻译 (RNA) 进行细胞调节的媒介。它们是用于自由基反应以及 H2、N2 和 CO2 活化的氧化还原催化剂，这些过程给工业带来了科学和经济挑战。铁硫中心为化学键、光谱学、结构和分子机制的基础研究提供了焦点，其功能对健康、医学和技术应用（包括可再生能源）具有许多影响。本次会议的具体目标是邀请来自多个领域的杰出演讲者和讨论领袖，主题范围从细菌酶的机制讨论到以铁硫簇生物合成途径异常为特征的新兴人类疾病的讨论。会议的健康相关性很明确，并将进行广泛讨论。自2010年以来，已经发现了三种由铁硫生物发生途径基因突变引起的新疾病，并且这些疾病都没有有效的治疗方法，包括弗里德赖希共济失调，一种以进行性神经退行性变和进行性心力衰竭为特征的疾病。需要新的治疗策略，化学家和医生首次结合在一起有可能促进该领域的进步。公共健康相关性：铁硫辅助因子对于几乎所有形式的生命都至关重要，并且在了解其特殊化学属性及其对新陈代谢的独特贡献方面已经取得了很大进展。在过去的十年里，人们已经清楚，未能正确合成铁硫簇和蛋白质会导致一系列罕见和严重的疾病，从婴儿乳酸酸中毒和癫痫发作到弗里德赖希共济失调（一种患者存活到成年早期的疾病）， ISCU 肌病，患者寿命正常，但骨骼肌功能明显受损。本次戈登研究会议对当前研究的讨论将明确与越来越多的独特疾病相关的重要问题，这是一个重要的机会，可以让研究人员聚集在一起，共同研究铁硫簇如何形成机制。组装和转移，这可能会导致铁硫簇缺乏症的新治疗策略。