GORDON RESEARCH CONFERENCE ON BASEMENT MEMBRANCES

本申请申请资助戈登基底膜会议特邀演讲者。这将是非常成功的一系列会议中的第十次，这些会议已经成为传播关于基底膜(BMS)结构和生物功能的新想法和信息的主要国际论坛。这些是复杂的三维细胞外结构，位于上皮细胞和间充质细胞的交界处，并包裹着间充质细胞。它们在大多数组织和器官的组织和功能中发挥着重要作用，如血管、肺、肾、皮肤、周围神经和肌肉。例如，基底膜有助于神经和肌肉骨骼系统中细胞的迁移和组织。基底膜成分或其受体编码基因的突变与人类严重的遗传性疾病(如皮肤大疱性表皮松解症、先天性肌营养不良、肾脏阿尔波特综合征)有关。基底膜获得性缺陷是糖尿病微血管病变的发病机制之一，也是麻风病或转移癌细胞等感染性病原体的进入部位。传统上，这次会议吸引了来自基础研究领域广泛领域的科学家，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，参与人类疾病转化研究或治疗的临床医生和科学家也参加了会议，这些疾病涉及肺、血管、皮肤、肾脏、骨骼、肌肉或免疫系统的BM成分。BM降解和周转的基础研究也引起了科学家们的兴趣，他们正在研究胚胎植入、乳腺或子宫退缩或癌症转移等动态过程。近年来，临床医生和制药和生物技术行业的科学家对BMS在伤口愈合、神经再生、炎症和组织修复中的作用进行了大量的研究，并产生了浓厚的兴趣。会议将就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提出各种不同的会议。脊椎动物和无脊椎动物BM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。最近关于骨髓基质影响细胞内信号通路、调节细胞存活以及与多肽生长因子激活的信号通路相互作用的机制的研究将是这次会议的特色，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，还将重点研究骨髓和细胞外基质功能的遗传学，人类骨髓疾病的特征，以及这些疾病的动物模型的建立。

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.