FASEB conference on Assembly of the Mitochondrial Respiratory Chain

描述：本提案寻求资金支持2007年FASEB线粒体呼吸链组装研究会议。主要目的是开创一个新的FASEB会议在线粒体生物学的一个迅速扩大的领域。会议将汇集科学家，从成熟的研究者到研究生，促进对线粒体生物学和人类疾病线粒体功能障碍的各个方面感兴趣的科学家之间的科学思想交流，以鼓励合作方法和跨领域刺激。请拨资金支持参加本次会议的受训者和初级教员。这次会议的时机很重要，因为对线粒体生物学和功能障碍的兴趣激增。近年来，在细胞器内的生理过程方面取得了重大进展，如形态动力学、蛋白质进入亚室、超复合体的识别和细胞器间通讯。从心肌病、肝病和神经退行性疾病的患者中收集了关于组装和调节的见解。大多数患者的生化缺陷是由于装配缺陷造成的，这可能导致无数的临床表型，包括共济失调、癫痫、失明、肌阵挛、肌张力障碍、铁母细胞性贫血、耳聋和糖尿病。因此，了解呼吸链的结构、组装和功能是一个非常重要的课题。会议将涉及线粒体的结构和动力学，呼吸链成分的组织，控制线粒体生物发生和功能的转录和翻译调节回路，线粒体-核和线粒体-细胞质串音，线粒体蛋白分类及其在疾病中的作用，个体呼吸复合物的组装，辅助因子的生物发生和嵴形成。线粒体呼吸链组装的新颖性在于其对呼吸链组分的生物发生和人类呼吸功能障碍的独特关注。没有其他会议尝试过这样做。2007年FASEB线粒体呼吸链大会旨在促进对线粒体呼吸和呼吸复合物生物发生的各个方面感兴趣的科学家之间的科学思想交流。近年来，人们对线粒体生物学的兴趣激增，因此这次会议的时机非常重要，因为它突出了线粒体动力学和形态学、内膜生物发生和细胞器间通讯方面的进展。线粒体在细胞凋亡、脂质代谢、能量产生、产热、铁代谢和其他分解代谢和合成代谢途径中发挥关键作用。线粒体功能障碍有助于许多遗传和获得性人类疾病和衰老过程。从心肌病、肝病和神经退行性疾病的患者中收集了关于组装和调节的见解。呼吸链组装缺陷可导致多种临床表型，包括共济失调、癫痫发作、失明、肌阵挛、肌张力障碍、铁母细胞性贫血、耳聋和糖尿病。因此，了解呼吸链的结构、组装和功能是一个非常重要的课题。线粒体呼吸链组装的新颖性在于其对呼吸链组分的生物发生和人类呼吸功能障碍的独特关注。没有其他会议尝试过这样做。

DESCRIPTION: This proposal seeks funds to support the 2007 FASEB Research Conference on Assembly of the Mitochondrial Respiratory Chain. The principal objective is to inaugurate a new FASEB conference on a rapidly expanding area of mitochondrial biology. The meeting will bring together scientists, ranging from established investigators to graduate students, to promote the exchange of scientific ideas among scientists interested in diverse aspects of mitochondrial biology and mitochondrial dysfunction in human disease, with a view to encouraging collaborative approaches and cross-field stimulation. Funds are requested to support trainees and junior faculty who would benefit from attendance at this conference. The timing of this conference is significant, as interest in mitochondrial biology and dysfunction has surged. Seminal advances have occurred in recent years on physiological processes within the organelle such as the dynamics of morphology, protein import into sub-compartments, identification of supercomplexes, and interorganelle communication. Insight has been gleaned from patients with cardiomyopathy, hepatopathy, and neurodegenerative disease on assembly and regulation. The biochemical defect in the majority of the patients results from a defect in assembly and this can lead to a myriad of clinical phenotypes ranging from ataxia, seizures, blindness, myoclonus, dystonia, sideroblastic anemia, deafness, and diabetes. An understanding of the structure, assembly and function of the respiratory chain is thus a highly significant topic. Sessions will deal with the structure and dynamics of the mitochondrion, the organization of respiratory chain components, transcriptional and translational regulatory circuits controlling mitochondrial biogenesis and function, mitochondrial-nuclear and mitochondrial- cytoplasm cross talk, mitochondrial protein sorting and its role in disease, assembly of individual respiratory complexes, cofactor biogenesis and cristae formation. The novelty of the Assembly of the Mitochondrial Respiratory Chain lies in the unique focus on biogenesis of the respiratory chain components and respiratory dysfunction in humans. No other meeting has attempted this. The 2007 FASEB conference Assembly of the Mitochondrial Respiratory Chain is designed to promote the exchange of scientific ideas among scientists interested in diverse aspects of mitochondrial respiration and biogenesis of the respiratory complexes. Interest in mitochondrial biology has surged in recent years, so the timing of this conference is significant in highlighting advances made in mitochondrial dynamics and morphology, inner membrane biogenesis and inter-organelle communication. Mitochondria play a key role in apoptosis, lipid metabolism, energy production, thermogenesis, iron metabolism and other catabolic and anabolic pathways. Mitochondrial dysfunction contributes to numerous inherited and acquired human diseases and the ageing process. Insight has been gleaned from patients with cardiomyopathy, hepatopathy, and neurodegenerative disease on assembly and regulation. Defects in assembly of the respiratory chain can lead to a myriad of clinical phenotypes ranging from ataxia, seizures, blindness, myoclonus, dystonia, sideroblastic anemia, deafness, and diabetes. Thus, an understanding of the structure, assembly and function of the respiratory chain is thus a highly significant topic. The novelty of the Assembly of the Mitochondrial Respiratory Chain lies in the unique focus on biogenesis of the respiratory chain components and respiratory dysfunction in humans. No other meeting has attempted this.