Gluconeogenic control of Dravet Syndrome

Dravet 综合征的糖异生控制

• 批准号: 10624665
• 负责人: Scott C Baraban
• 金额: $ 15.89万
• 依托单位: UNIVERSITY OF COLORADO DENVER
• 依托单位国家: 美国
• 财政年份: 2020
• 资助国家: 美国
• 起止时间: 2020-06-01 至 2025-05-31
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/10624665
• 关键词: Administrative Supplement; Biological Assay; Childhood; Defect; Disease; Disease model; Epilepsy; Genetic; Genus Hippocampus; Goals; Larva; Measures; Metabolic; Mitochondria; Modeling; Pathway interactions; Research; Seizures; Technology; Time; Zebrafish; behavioral impairment; dravet syndrome; extracellular; innovation; insight; instrument; metabolic abnormality assessment; novel; parent project

The goal of this administrative supplement is to request the purchase of a critical instrument (Seahorse XF24e, Agilent Technologies) which is necessary for the continuation and completion of the project. To achieve the major aims and goals of the parent project are to study metabolic defects in a devastating neurodevelopmental disease known as Dravet Syndrome. A key assay that we have developed for this project is assessment of metabolic defects in a zebrafish model of the disease. The assay involves the use of an extracellular flux analyzer that measures mitochondrial and glycolytic functions in real time in live zebrafish larvae. The instrument is requested to replace a new version of a previous model which has been retired by the company.

本行政补充文件的目的是请求购买一台关键仪器（Seahorse XF24 e， 安捷伦科技公司），这是必要的继续和完成项目。实现 母项目的主要目的和目标是研究破坏性神经发育的代谢缺陷， 被称为Dravet综合征的疾病。我们为该项目开发的一个关键检测方法是评估 斑马鱼疾病模型中的代谢缺陷。该测定涉及使用细胞外通量分析仪 在活体斑马鱼幼鱼中真实的测量线粒体和糖酵解功能。仪器 要求更换该公司已退役的旧型号的新版本。