INTERNATIONAL CONFERENCE ON THE CEROID-LIOPFUSCINOSES

要求提供资金，以部分支持第七国际 神经元蜡样脂褐质病（Neuronal Ceroid-Lipofuscinoses，NCL） 这次会议 已成为介绍NCL新数据的主要论坛 research. 神经元蜡样脂褐质病（Batten病）是一组 遗传性进行性神经系统疾病的特点是运动 感觉退化、视力衰退、严重智力迟钝，以及 癫痫发作 这种疾病的病理特征是 被称为蜡样物质的自发荧光脂色素在 大脑和其他组织。 它们是非常常见的神经遗传性疾病 影响婴幼儿。一些估计表明， 在美国，这种疾病比所有 鞘脂综合征。 虽然疾病实体已经被 100多年来，生物化学缺陷仍然存在 很大程度上是未知的，没有可用的治疗方法。前六 关于这一主题的国际会议引发了许多 该领域的合作研究活动， 来自世界各地的研究人员。 目前，正在取得快速进展。 在理解生物化学缺陷，基因定位， 基因组织和这些疾病的分子诊断。 的 计划中的会议将汇集来自不同国家的科学家 学科和研究兴趣，以加强有关这些信息 特别强调了解发病机制的进展 的障碍。 所要求的资金专门用于 以及受邀演讲者的住宿费用。

Funds are requested for partial support of the Seventh International Conference on the Neuronal Ceroid-Lipofuscinoses (NCL). This conference has become the primary forum for the presentation of new data on NCL research. Neuronal ceroid-lipofuscinoses (Batten Disease) are a group of inherited, progressive neurological disorders characterized by motor sensory deterioration, visual failure, severe mental retardation, and seizures. The pathological hallmark of this disorder is the accumulation of autofluorescent lipopigments known as ceroids in the brain and other tissues. They are very common neurogenetic disorders affecting infants and children. Some estimates indicate that in the United States, the disorders are more prevalent than all the sphingolipidoses combined. Although the disease entities have been recognized for more than 100 years, the biochemical defects remain largely unknown and no treatments are available. The previous six international conferences on this subject have sparked much collaborative research activity in this field bringing together researchers from across the world. Currently rapid progress is being made in understanding the biochemical defects, genetic localizations, gene organizations and molecular diagnosis of these disorders. The planned conference will bring together scientists of different disciplines and research interests to enhance information about these advances with a particular emphasis on understanding the pathogenesis of the disorders. The funds requested are specifically for the travel and lodging expenses of the invited speakers.