Mortensen P B
Biochim Biophys Acta. 1986 Aug 14;878(1):14-9. doi: 10.1016/0005-2760(86)90338-3.
A combined gas chromatographic-mass spectrometric method (selected ion monitoring) to determine C6-C10-dicarboxylic acids in liver and kidney tissue is reported. Alterations in tissue concentrations of the dicarboxylic acids were reflected in urinary excretions, i.e., diabetic rats with 'ketotic dicarboxylic aciduria' had corresponding elevated concentrations of short-chain dicarboxylic acids in liver and kidney tissue. Stimulation of the enzymes of fatty acid oxidation by clofibrate was, as a sole event, not sufficient to cause elevated tissue concentrations of dicarboxylic acids, nor did it result in dicarboxylic aciduria, probably because of a relative lack in substrate (fatty acids) compared to the diabetic ketotic state, where lipolysis is increased. These results strongly indicate that 'ketotic dicarboxylic aciduria' parallels the activity of the lipid metabolism at cellular level, and that it is not just a matter of renal handling.
本文报道了一种用于测定肝脏和肾脏组织中C6 - C10二羧酸的气相色谱 - 质谱联用方法（选择离子监测）。二羧酸组织浓度的变化反映在尿液排泄中，即患有“酮症性二羧酸尿症”的糖尿病大鼠肝脏和肾脏组织中短链二羧酸的浓度相应升高。仅用氯贝丁酯刺激脂肪酸氧化酶不足以导致组织中二羧酸浓度升高，也不会导致二羧酸尿症，这可能是因为与糖尿病酮症状态相比，底物（脂肪酸）相对缺乏，而糖尿病酮症状态下脂肪分解增加。这些结果有力地表明，“酮症性二羧酸尿症”与细胞水平的脂质代谢活动平行，而不仅仅是肾脏处理的问题。
