2004 Intermediate Filaments Gordon Conference

描述（由申请人提供）： 本提案的目的是筹集资金，资助参加第八届戈登中间纤维研究会议的与会者的旅费、登记费和住宿费，该会议将于2004年8月8日至13日在联合王国牛津大学女王学院举行。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>67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性疾病（例如，皮肤、口腔和眼睛起泡疾病;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）、脂肪营养不良和过早老化性梅毒。IF蛋白以分化和环境依赖的方式实现其他功能，包括促进特定的细胞结构、对损伤和其他形式的应激的组织响应、对凋亡信号的响应、信号传导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。2004年会议的计划已经完成，将主要集中在这些蛋白质的广泛疾病方面，它们在细胞信号传导中的新作用，与它们相互作用的相关蛋白质的扩大列表，新的结构发现和潜在的新治疗方法。已安排的具体会议是：1）IF的结构，组装和生物物理学; 2）核纤层生物学和疾病; 3）IF和细胞凋亡; 4）角蛋白调节和疾病; 5）IF，应激和信号传导; 6）神经元IF生物学和疾病; 7）IF相关蛋白质：从生物学到疾病; 8）我们需要知道的IF剩下了什么？9)IF研究的新视野。

DESCRIPTION (provided by applicant): The purpose of this proposal is to generate funds to support travel, registration and lodging for participants in the 8th Gordon Research Conference on Intermediate Filaments, which will be held August 8- 13, 2004 at Queen's College, Oxford, United Kingdom. Intermediate Filaments (IFs) are major components of the cytoskeleton and nucleoskeleton in higher eukaryotes. In the public draft of the human genome, there are >67 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 disorders involving epithelia (e.g., skin, oral and eye blistering diseases; liver disorders), muscle (e.g., cardiomyopathies; muscular dystrophy), neural tissue (e.g., amyotrophic lateral sclerosis; Alexander's diseases), lipodystrophies, and premature aging sydromes. IF proteins fulfill other functions in a differentiation and context-dependent fashion, including promoting specific cytoarchitecture, tissue response to injury and other forms of stress, response to apoptotic signals, signaling, 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 2004 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, 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) Structure, Assembly and Biophysics of IFs; 2) Lamin Biology and Diseases; 3) IFs and Apoptosis; 4) Keratin Regulation and Diseases; 5) IFs, Stress and Signaling; 6) Neuronal IF Biology and Diseases; 7) IF Associated Proteins: from Biology to Disease; 8) What's Left of IFs that we need to know? 9) New Horizons in IF Studies.