TRACER KINETIC STUDIES OF GALACTOSE METABOLISM IN HEREDITARY GALACTOSEMIA

遗传性半乳糖血症中半乳糖代谢的示踪动力学研究

• 批准号: 6219804
• 负责人: Gerard Thomas Berry
• 金额: $ 0.06万
• 依托单位: CHILDREN'S HOSP OF PHILADELPHIA
• 依托单位国家: 美国
• 财政年份: 1998
• 资助国家: 美国
• 起止时间: 1998-12-01 至 1999-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6219804
• 关键词: carbohydrate balance; carbohydrate metabolism; clinical research; folate; galactose; galactosemias; human subject; metabolism disorder chemotherapy; pharmacokinetics; progesterone; uridine

The first aim of this study is the determination of the endogenous galactose, galactitol and galactonate pool sizes in the galactosemic patient, all of these being factors in the body galactose burden. The second aim is the determination of the in vivo galactose oxidation rate in the galactosemic patient. The third aim is the determination whether GALT activity can be modulated in vivo by pharmocologic administration of uridine, folate or progesterone.

本研究的第一个目的是测定半乳糖血症患者的内源性半乳糖、半乳糖醇和半乳糖酸盐池的大小，所有这些都是体内半乳糖负荷的因素。 第二个目的是测定半乳糖血症患者体内半乳糖氧化速率。 第三个目的是确定GALT活性是否可以通过尿苷、叶酸或孕酮的药理学给药在体内调节。