枫糖尿症患者血液中支链氨基酸与支链α-酮酸的相关性
Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease.
作者信息
Langenbeck U, Wendel U, Mench-Hoinowski A, Kuschel D, Becker K, Przyrembel H, Bremer H J
出版信息
Clin Chim Acta. 1978 Sep 1;88(2):283-91. doi: 10.1016/0009-8981(78)90433-3.
PMID:699323
Abstract
In 62 blood samples from 3 patients with classical maple syrup urine disease and from one patient with a variant form, a close linear correlation was found between levels of branched chain amino acids and their corresponding alpha-keto acids. Keto acids were determined as O-trimethylsilyl quinoxalinols by gas chromatography with a nitrogen-selective detector.
摘要
在来自3例典型枫糖尿症患者和1例变异型患者的62份血样中，发现支链氨基酸水平与其相应的α-酮酸水平之间存在密切的线性相关性。酮酸通过带有氮选择性检测器的气相色谱法测定为O-三甲基硅基喹喔啉醇。
Zotero 插件