The Progeria Research Foundation International Progeria Workshop

描述（由申请人提供）： Hutchinson-Gilford早老症（英语：Hutchinson-Gilford Progeria Syndrome）是一种罕见的、节段性的、过早衰老的综合征，其中儿童在平均13岁时死于严重的过早心脏病（动脉粥样硬化）。早衰症研究基金会（PRF）的使命是通过研究和教育（www.progeriaresearch.org）发现早衰症及其衰老相关疾病的原因、治疗和治愈。NIH一直是这一进程中不可或缺的一部分，在我们之前的三次关于早衰症的科学会议中提供了必要的参与。该论坛目前正在召集一批国际知名的科学家，于2005年11月3日至5日在马萨诸塞州波士顿举行第四次讲习班。我们的目标是促进对早衰症及其衰老相关疾病的研究;为科学家提供一个大学环境，通过口头报告，海报会议，一对一讨论以及与患有早衰症的儿童和家庭会面的机会，介绍早衰症领域的最新实验结果。这种环境将促进未来研究的新思路，并促进新的科学合作。过去的PRF研讨会已经产生了导致科学突破的合作，例如发现导致早衰症的基因，早衰症细胞核的组织学和功能表征，以及对修复早衰症细胞损伤的见解。早衰症是细胞衰老和动脉粥样硬化研究的最新和最有前途的领域之一，主要是因为它的遗传缺陷最近才被发现。演讲者包括衰老，心脏病，遗传学和核纤层蛋白病领域的顶尖科学家，这次科学会议有望影响这个年轻和快速发展的研究领域。 自上次研讨会以来已发表和未发表的数据包括以下领域的重大进展，将对此进行讨论：早老症的细胞学特征、核纤层蛋白A的生物化学及蛋白质缺陷产物（早老症）、衰老和早老症、新的小鼠模型、临床自然史研究、普通人群中心血管疾病的线索、包括RNA干扰在内的治疗策略、遗传疗法、和药物治疗。演讲者将包括与早衰症，心脏病和衰老相关领域的专家。了解早衰症不仅有助于这些儿童，而且可能为治疗数百万患有与自然衰老过程相关的心脏病和中风的成年人提供关键。

DESCRIPTION (provided by applicant): Hutchinson-Gilford Progeria Syndrome (Progeria) is a rare, segmental, premature aging syndrome in which children die of severe premature heart disease (atherosclerosis) at an average age of thirteen years. The mission of The Progeria Research Foundation (PRF) is to discover the cause, treatment and cure for Progeria and its aging-related disorders, through research and education (www.progeriaresearch.org). The NIH has been an integral part of this process, providing essential participation in each of our prior three scientific meetings on Progeria. PRF is now assembling a group of internationally recognized scientists in Boston, Massachusetts on November 3-5, 2005 for its fourth workshop. Our goal is to promote research on Progeria and its aging-related disorders; to provide a collegiate environment for scientists to present recent experimental findings in the field of Progeria through oral presentations, poster sessions, one-on-one discussions, and the opportunity to meet with children and families living with Progeria. This environment will promote new ideas for future research and foster new scientific collaborations. PRF workshops in the past have generated collaborations that have led to scientific breakthroughs, such as the discovery of the gene that causes Progeria, histological and functional characterization of the nucleus in Progeria cells, and insight into repairing cellular damage in Progeria. Progeria is one of the newest and most promising areas of research for cellular aging and atherosclerosis, primarily because its genetic defect has only recently been identified. With speakers that include leading scientists in the fields of aging, heart disease, genetics, and laminopathies, this scientific meeting promises to influence this young and fast-growing field of study. Published and unpublished data since the last workshop includes significant advances in the following areas, which will be discussed: cellular characteristics of Progeria, biochemistry of Lamin A and the defective protein product in Progeria (progerin), aging and Progeria, new mouse models, clinical natural history studies, clues to cardiovascular disease in the general population, treatment strategies including RNA interference, genetic therapies, and drug treatment. Speakers will include experts in areas related to Progeria, heart disease and aging. Understanding Progeria will not only help these children, but may provide keys for treating millions of adults with heart disease and stroke associated with the natural aging process.