Intermediate Filaments 2008 Gordon Research Conference

描述（由申请人提供）：本提案是一份资金申请，用于支持第10届戈登中间纤维研究会议与会者的旅行和注册，该会议将于2008年9月7日至2009年9月10日举行。12，2008年英国牛津大学马格达兰学院。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图中，有65个编码IF形成多肽的功能基因。这些基因以细胞特异性方式调节，并且在哺乳动物基因组中高度保守。IF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蛋白的缺陷是大量涉及上皮细胞的遗传决定的罕见和常见疾病的基础（例如，皮肤、口腔、眼睛和肝脏疾病），肌肉（例如，心肌病;肌营养不良），神经组织（例如，肌萎缩性侧索硬化;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实现其他功能，包括对凋亡信号的响应、蛋白质靶向亚细胞区室、细胞信号传导、亚细胞器功能和核结构以及基因表达。这个戈登研究会议（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，该研究中心促进了新思想、工具和试剂的自由交流，并促进了富有成效的合作。2008年版的会议计划已经完成，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，与它们相互作用的相关蛋白质的扩大列表，新的结构发现，以及潜在的新治疗方法。已安排的具体会议有：1）通过IF支架的信号传导; 2）细胞骨架的分子机制; 3）核纤层蛋白和核纤层相关蛋白; 4）核纤层病; 5）神经系统的IF系统; 6）IF蛋白突变引起的神经病理学; 7）角蛋白在发育和疾病中的作用; 8）肌肉功能和结蛋白病; 9）将IF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.