3-甲基巴豆酰甘氨酸在3-羟基-3-甲基戊二酸尿症中的排泄情况。
3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria.
作者信息
Wysocki S J, Hähnel R
出版信息
Clin Chim Acta. 1978 May 16;86(1):101-8. doi: 10.1016/0009-8981(78)90464-3.
PMID:657530
Abstract
- 3-Methylcrotonylglycine was identified in urine from an infant with 3-hydroxy-3-methylglutaric aciduria. 2. The concentration of 3-methylcrotonylglycine in urine was approximately one sixth of that of the other metabolite of 3-methylcrotonyl-CoA, 3-hydroxyisovaleric acid. 3. The presence of both metabolites in the infant's urine indicates an inhibition of 3-methylcrotonyl-CoA carboxylase activity in tissues of the infant.
摘要
- 在一名患有3-羟基-3-甲基戊二酸尿症的婴儿尿液中发现了3-甲基巴豆酰甘氨酸。2. 尿液中3-甲基巴豆酰甘氨酸的浓度约为3-甲基巴豆酰辅酶A的另一种代谢产物3-羟基异戊酸浓度的六分之一。3. 婴儿尿液中这两种代谢产物的存在表明婴儿组织中3-甲基巴豆酰辅酶A羧化酶活性受到抑制。
Zotero 插件