苯甲酰丙氨酸：在接受苯甲酸钠治疗的高氨血症患者中检测和鉴定与苯甲酸结合的丙氨酸

Benzoylalanine: detection and identification of an alanine conjugate with benzoic acid in hyperammonemic patients treated with sodium benzoate.

作者信息

Shinka T, Inoue Y, Kuhara T, Matsumoto M, Matsumoto I

出版信息

Clin Chim Acta. 1985 Oct 15;151(3):293-300. doi: 10.1016/0009-8981(85)90092-0.

Abstract

Urine of four hyperammonemic patients who were treated with large amounts of sodium benzoate were analyzed. Gas chromatographic and gas chromatograph-mass spectrometric analysis showed the presence of large amounts of benzoylalanine in addition to much hippuric acid. Benzoylalanine was identified by comparison with an authentic standard by means of its mass spectrum and the gas chromatographic retention time of its trimethylsilyl derivative. This abnormal metabolite is thought to be derived from the conjugation of benzoyl CoA with alanine. It is suggested that the excretion of benzoylalanine results from a reduction in the liver free glycine level caused by hippuric acid synthesis.

摘要

对四名接受大量苯甲酸钠治疗的高氨血症患者的尿液进行了分析。气相色谱和气相色谱 - 质谱分析表明，除了大量马尿酸外，还存在大量苯甲酰丙氨酸。通过其质谱及其三甲基硅烷基衍生物的气相色谱保留时间与 authentic standard 进行比较，鉴定出了苯甲酰丙氨酸。这种异常代谢产物被认为是由苯甲酰辅酶A与丙氨酸结合产生的。有人提出，苯甲酰丙氨酸的排泄是由于马尿酸合成导致肝脏游离甘氨酸水平降低所致。