Intermediate Filaments 2008 Gordon Research Conference

描述（由申请人提供）：本提案为参加将于2008年9月7日至9月12日在英国牛津马格达伦学院举行的第十届戈登中间细丝研究会议的与会者申请旅费和注册资金。中间丝是高等真核生物细胞骨架和核骨架的主要组成部分。在公开的人类基因组草案中，有65个功能基因编码if形成多肽。这些基因以细胞特异性方式调控，在哺乳动物基因组中高度保守。IFs的一般功能是赋予细胞和组织机械弹性，以承受各种类型的物理和非物理应力。IF蛋白缺陷是大量由基因决定的罕见和常见疾病的基础，包括上皮（如皮肤、口腔、眼睛和肝脏疾病）、肌肉（如心肌病、肌肉萎缩症）、神经组织（如肌萎缩性侧索硬化症、亚历山大病）、脂肪营养不良和早衰综合征。IF蛋白以分化和环境依赖的方式完成其他功能，包括对凋亡信号的反应，亚细胞区室的蛋白靶向，细胞信号传导，亚细胞细胞器功能和核结构以及基因表达。戈登研究会议（GRC）是唯一一个专门讨论IF生物学的定期会议。它汇集了来自世界各地的初级和高级参与者，他们从功能，调节，结构和疾病相关的角度研究IF蛋白。传统上，GRC促进了新思想、工具和试剂的自由交流，并促进了富有成效的合作的建立。2008年会议的计划已经完成，将主要关注这些蛋白质的广泛疾病方面，它们在细胞信号传导和细胞器功能中的新作用，它们相互作用的相关蛋白质的扩大列表，新的结构发现和潜在的新治疗方法。已安排的具体会话有：1)通过中频支架发出信号；2)细胞骨架的分子机制；3)层粘连蛋白和层粘连蛋白；4)层压病；5)神经系统的中频系统；6) IF蛋白突变引起的神经病变；7)发育和疾病中的角蛋白；8)肌肉功能和末梢病变；将中频系统整合到细胞生理学中的因素。

DESCRIPTION (provided by applicant): This proposal is a request for funds to support travel and registration for participants in the 10th Gordon Research Conference on Intermediate Filaments, which will be held Sept 7, 2008 to Sept. 12, 2008 Magdalan College, Oxford, UK. Intermediate Filaments (IFs) are major components of the cytoskeleton and nucleoskeleton in higher eukaryotes. In the public draft of the human genome, there are 65 functional genes encoding IF-forming polypeptides. These genes are regulated in a cell-specific manner and are highly conserved in mammalian genomes. A general function of IFs is to endow cells and tissues with mechanical resilience to withstand various types of physical and non-physical stresses. Defects in IF proteins underlie a vast number of genetically determined rare and common disorders involving epithelia (e.g., skin, oral, eye and liver disorders), muscle (e.g., cardiomyopathies; muscular dystrophy), neural tissue (e.g., amyotrophic lateral sclerosis; Alexander disease), lipodystrophies, and premature aging syndromes. IF proteins fulfill other functions in a differentiation and context-dependent fashion, including the response to apoptotic signals, protein targeting to subcellular compartments, cell signaling, subcellular organelle function and nuclear architecture and gene expression. This Gordon Research Conference (GRC) represents the only regular meeting devoted to IF biology. It brings together participants of junior and senior rank from all over the world, who study IF proteins from functional, regulatory, structural, and disease-related perspectives. This GRC has traditionally fostered a free-flowing exchange of novel ideas, tools and reagents, and facilitated the establishment of productive collaborations. The Program for the 2008 edition of the Conference has been finalized and will have a major focus on the broad disease aspect of these proteins, their emerging role in cell signaling and organelle function, the enlarging list of associated proteins they interact with, new structural findings, and potential novel approaches for therapy. The specific sessions that have been scheduled are: 1) Signaling via the IF scaffolds; 2) Molecular mechanism of the cytoskeleton; 3) Lamins and lamina-associated proteins; 4) Laminopathic diseases; 5) IF systems of the nervous system; 6) Neuropathologies caused by mutations in IF proteins; 7) Keratins in development and disease; 8) Muscle function and desminopathies; 9) Factors integrating IF systems into cellular physiology.