异戊酰葡萄糖醛酸，一种异戊酸血症中的新型尿液代谢物。重排反应导致的鉴定问题。

Isovalerylglucuronide, a new urinary metabolite in isovaleric acidemia. Identification problems due to rearrangement reactions.

作者信息

Dorland L, Duran M, Wadman S K, Niederwieser A, Bruinvis L, Ketting D

出版信息

Clin Chim Acta. 1983 Oct 31;134(1-2):77-83. doi: 10.1016/0009-8981(83)90186-9.

Abstract

Isovaleryl-beta-D-glucuronide, a new metabolite in the urine of patients with isovaleric acidemia, is described. Its gas chromatographic and mass spectrometric parameters are presented. In alkaline solution this glucuronide exhibited intramolecular rearrangements, resulting in isomers bearing the acyl moiety on C-2, C-3 and C-4. The isomers showed similar mass spectra but different positions on the gas chromatogram. In the index patient isovalerylglucuronide was a main metabolite, but the excretion was a transient phenomenon. Only traces of isovalerylglucuronide could also be detected in the urine of three other patients with isovaleric acidemia. The significance of this metabolite for the detoxication of isovalerate in isovaleric acidemia is discussed.

摘要

描述了异戊酰基-β-D-葡萄糖醛酸苷，这是异戊酸血症患者尿液中的一种新代谢物。给出了其气相色谱和质谱参数。在碱性溶液中，这种葡萄糖醛酸苷会发生分子内重排，产生在C-2、C-3和C-4上带有酰基部分的异构体。这些异构体具有相似的质谱，但在气相色谱图上的位置不同。在索引患者中，异戊酰葡萄糖醛酸苷是主要代谢物，但排泄是一种短暂现象。在另外三名异戊酸血症患者的尿液中也仅检测到痕量的异戊酰葡萄糖醛酸苷。讨论了这种代谢物在异戊酸血症中对异戊酸解毒的意义。