GORDON RESEARCH CONFERENCE ON BASEMENT MEMBRANCES

该申请要求提供资金，以支持戈登地下室膜会议的特邀发言人。 这将是一系列非常成功的会议中的第十次，这些会议已成为传播有关基底膜（Bms）结构和生物功能的新思想和信息的主要国际论坛。 这些是位于上皮细胞和间充质细胞界面并包裹间充质细胞的复杂的三维细胞外结构。 它们在大多数组织和器官的组织和功能中发挥重要作用，例如血管、肺、肾、皮肤、外周神经和肌肉。 例如，基底膜促进神经和肌肉骨骼系统中细胞的迁移和组织。 编码基底膜成分或其受体的基因突变与人类严重遗传性疾病（例如皮肤大疱性表皮病、先天性肌营养不良症、Alport肾病综合征）相关。 基底膜的获得性缺陷有助于糖尿病微血管疾病的发病机制，并作为感染因子（如麻风病或转移性癌细胞）的进入部位。传统上，该会议吸引了来自基础研究各个领域的科学家，包括蛋白质和碳水化合物结构、基因表达、细胞和发育生物学以及神经生物学。 此外，参与转化研究或治疗涉及肺、血管、皮肤、肾、骨、肌肉或免疫系统BM成分的人类疾病的临床医生和科学家也参加了会议。BM降解和周转的基础研究也使研究动态过程如胚胎发育、乳腺或子宫退化或癌症转移的科学家感兴趣。 近年来，制药和生物技术行业的临床医生和科学家对研究Bms在伤口愈合、神经再生、炎症和组织修复中的作用产生了浓厚的兴趣。 会议将提出关于基底膜和细胞外基质（ECM）结构，生物合成，组装周转和功能的基础科学的各种混合会议。 BM在脊椎动物和无脊椎动物中功能的比较研究，以及BM和ECM在胚胎发育中的作用也将纳入该计划。最近的工作机制BM影响细胞内信号通路，调节细胞存活，并与多肽生长因子激活的信号通路相互作用将是会议的特点，因为这些研究对理解正常功能和疾病过程具有广泛的意义。 此外，重点将放在BM和ECM功能的遗传学研究，人类BM疾病的表征，以及这些疾病的动物模型的产生。

This application requests funding for the support of invited speakers for the Gordon Conference on Basement Membranes. This will be the tenth in a highly successful series of conferences, which have become a major international forum for the dissemination of new ideas and information about the structure and biological functions of basement membranes (Bms). These are complex, three-dimensional, extracellular structures at the interface of epithelial and mesenchymal cells and enveloping mesenchymal cells. They play important roles in the organization and function of most tissues and organs, e.g. blood vessels, lung, kidney, skin, peripheral nerve, and muscle. For example, basement membranes facilitate the migration and organization of cells in the nervous and musculoskeletal systems. Mutations in genes encoding basement membrane components or their receptors are associated with severe inherited disorders in humans (e.g. epidermolysis bullosa of skin, congenital muscular dystrophy, Alport's syndrome of kidney). Acquired defects in basement membranes contribute to the pathogenesis of diabetic microvascular disease, and serve as entry sites for infectious agents, such as leprosy, or metastatic cancer cells. Traditionally, the conference has attracted scientists from a wide range of fields in basic research, including protein and carbohydrate structure, gene expression, cell and developmental biology, and neurobiology. In addition, it has been attended by clinicians and scientists involved in translational research or treatment of human disorders involving BM components of lung, blood vessels, skin, kidney, bone, muscle or the immune system. Basic studies of BM degradation and turnover also interest scientists investigating dynamic processes such as embryo implanatation, involution of the mammary gland or uterus, or cancer metastases. In recent years there has been substantial attendance and interest from clinicians and scientists in the pharmaceutical and biotechnology industries studying the role of Bms in wound healing, nerve regeneration, inflammation and tissue repair. The Conference will present a diverse mixture of sessions on the basic science of basement membrane and extracellular matrix (ECM) structure, biosynthesis, assembly turnover and functions. Comparative studies of BM function in vertebrates and invertebrates, and the role of BM and ECM in embryonic development will also be incorporated into the program. Recent work on mechanism by which BMs affect intracellular signaling pathways, regulate cell survival, and interact with polypeptide growth factor-activated signaling paths will be features of the meeting, as these studies have broad implications for understanding normal function and disease processes. In addition, emphasis will be given to studies on the genetics of BM and ECM function, the characterization of human BM diseases, and the generation of animal models for these diseases.