2012 Iron Sulfur Enzymes Gordon Research Conference

描述(由申请人提供)：本申请请求部分支持将于2012年6月10-15日在马萨诸塞州南哈德利的芒特霍利奥克学院举行的戈登研究会议系列会议的一部分，会议将以广泛和长期的目标汇聚世界领先的铁硫集群专家，以独特的跨学科方式专注于酶机制、生物发生、调节和人类疾病中的作用。最初于1994年举办，专注于固氮酶及其独特的铁硫化学，关于铁硫簇的知识在过去20年中呈指数级增长，2006年，会议名称相应地更名为戈登铁硫酶会议。这次会议涉及面很广，有关于单细胞生物、动物和植物的许多铁硫酶的会议，这些会议为如何合成和利用这些多功能蛋白质提供了重要的见解。含有铁-硫辅助因子的蛋白质具有多种生物学功能， 电子传递、酸碱催化，以及在哪里它们是通过转录(DNA)或翻译(RNA)进行细胞调节的试剂。它们是氧化还原催化剂，用于基于自由基的反应以及H2、N2和CO2的活化，这些过程给工业带来了科学和经济上的挑战。铁硫中心是化学键、光谱、结构和分子机理等基础研究的焦点，其功能对健康、医学和技术应用，包括可再生能源具有重要意义。这次会议的具体目的是包括来自许多领域的杰出演讲者和讨论领袖，主题将从细菌酶的机械性讨论到以铁-硫簇生物发生途径异常为特征的一类新兴人类疾病的讨论。这次会议与健康的关系是明确的，并将得到广泛讨论。自2010年以来，已经发现了三种由铁-硫生物发生途径中的基因突变引起的新疾病，其中任何一种疾病都没有有效的治疗方法，包括Friedreich共济失调，这是一种以进行性神经退化和进行性心力衰竭为特征的疾病。需要新的治疗策略，首次将化学家和医生混合在一起有可能催化该领域的进展。 与公共健康相关：铁硫辅助因子对几乎所有形式的生命都是必不可少的，在了解它们的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，已经清楚的是，如果不能正确地合成铁硫簇和蛋白质，就会导致一系列罕见而严重的疾病，从婴儿乳酸血症和癫痫到Friedreich共济失调，这种疾病患者可以存活到成年早期，以及ISCU肌病，在这种疾病中，患者的寿命正常，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定义与越来越多的独特疾病相关的重要问题和问题，这是一个至关重要的机会，可以让研究人员聚集在一起，开发铁-硫簇如何组装和转移的机制洞察力，这可能会导致铁硫簇缺乏症的新治疗策略。

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.