INTERNATIONAL CONFERENCE ON THE CEROID-LIOPFUSCINOSES

蜡质-脂褐质国际会议

• 批准号: 2723292
• 负责人: Michael Bennett
• 金额: $ 2.15万
• 依托单位: UT SOUTHWESTERN MEDICAL CENTER
• 依托单位国家: 美国
• 财政年份: 1998
• 资助国家: 美国
• 起止时间: 1998-06-13 至 1998-06-16
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/2723292
• 关键词: INTERNATIONAL; CONFERENCE; CEROID; LIOPFUSCINOSES

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.

请求资金用于部分支持第七国际 神经元蜡样质脂褐质沉积症 (NCL) 会议。 本次大会 已成为展示 NCL 新数据的主要论坛 研究。 神经元蜡样脂褐质沉积症（巴顿病）是一组 以运动为特征的遗传性、进行性神经系统疾病 感觉减退、视力障碍、严重智力障碍 癫痫发作。 这种疾病的病理特征是 被称为蜡质的自发荧光脂色素在 脑和其他组织。 它们是非常常见的神经遗传性疾病 影响婴儿和儿童。一些估计表明，在 在美国，这些疾病比所有疾病都更为普遍 合并鞘脂沉积症。 尽管疾病实体已 100多年来，生化缺陷仍然存在 很大程度上未知，也没有可用的治疗方法。前六名 关于这一主题的国际会议引起了广泛关注 该领域的合作研究活动汇集了 来自世界各地的研究人员。 目前进展迅速 是为了了解生化缺陷、遗传定位、 这些疾病的基因组织和分子诊断。 这 计划中的会议将汇集不同领域的科学家 学科和研究兴趣，以增强有关这些的信息 特别强调了解发病机制的进展 的疾病。 所申请的资金专门用于旅行 以及受邀演讲者的住宿费用。