2010 Intermediate Filaments Gordon Research Conference

描述(申请人提供)：中间丝(IF)是真核细胞骨架和核骨架的重要结构元素。它们代表着最大的基因家族之一，有70多个基因编码不同的IF，这是组织类型特有的，并以发育调节的方式表达。它们为细胞骨架提供了稳定性和机械弹性。最近对它们与其他细胞骨架和核骨架元素相互作用的研究为了解它们在许多不同组织中的细胞结构和功能中的独特作用提供了重要的见解。最近的生物医学研究表明，IF中的缺陷是导致许多人类疾病的原因，从水疱性皮肤病到肌肉营养不良和早衰疾病。这些发现，再加上最近的研究表明，角蛋白对蛋白质生物合成的调控和Lamins对转录的控制，需要对IF蛋白质主要是结构蛋白的经典观点进行修订，并有必要将IF研究与其他对蛋白质折叠、炎症和信号转导感兴趣的研究团体联系起来。即将举行的IF蛋白质GRC旨在向来自不同领域的研究人员展示现有的IF研究小组，提供一个机会来研究复杂的问题，如IF在心脏、骨骼肌和神经退行性疾病中的作用。

DESCRIPTION (provided by applicant): Intermediate Filaments (IFs) are important structural elements of the eukaryotic cytoskeleton and nucleoskeleton. They represent one of the largest gene families, with over 70 genes coding for different IF that are tissue type specific and expressed in a developmentally regulated manner. They provide stability as well as mechanical resilience to the cell cytoskeleton. Recent studies on their interaction with other cytoskeletal and nucleoskeletal elements provide important insights into their unique role in structure and function of cells in many different tissues. Recent biomedical research has demonstrated that defects in IF are responsible for a great many human diseases ranging from blistering skin diseases to muscular dystrophy and premature aging disorders. These findings, combined with recent studies showing the regulation of protein biosynthesis by keratins and transcription control by lamins demands a revision of the classical view of IF proteins as primarily structural proteins and necessitates linking IF research to other research communities with an interest in protein folding, inflammation and signaling. The upcoming GRC on IF proteins is designed to expose established IF research groups to researchers from different fields, offering an opportunity to investigate complex problems such as the role of IF in cardiac, skeletal muscle and neurodegenerative diseases.