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要求购买关键仪器（Seahorse XF24e、 安捷伦科技），这对于项目的继续和完成是必要的。为了实现 父项目的主要目的和目标是研究破坏性神经发育中的代谢缺陷 称为 Dravet 综合症的疾病。我们为这个项目开发的一个关键分析是评估 该疾病的斑马鱼模型中的代谢缺陷。该测定涉及使用细胞外通量分析仪 实时测量活体斑马鱼幼虫的线粒体和糖酵解功能。该仪器是 要求更换公司已退役的旧型号的新版本。