Millington D S, Roe C R, Maltby D A
Biomed Mass Spectrom. 1984 May;11(5):236-41. doi: 10.1002/bms.1200110508.
Acylcarnitines, a biologically important group of metabolites which have thus far eluded characterization by mass spectrometry, exhibit very intense fast atom bombardment mass spectra dominated by parent cations. The combination of high resolution selected ion detection and daughter ion analysis using the linked scan at constant B/E ratio has enabled the unequivocal identification of the acylcarnitines in the urine of children with propionic acidemia, methylmalonic aciduria and Reye's Syndrome. Quantitative analysis of acetylcarnitine and propionylcarnitine in selected samples was accomplished by isotope dilution, utilizing (2H3)acetyl- and (2H5)propionylcarnitines as internal standards. These techniques will allow assessment of the therapeutic use of L-carnitine in these disorders.
酰基肉碱是一类具有重要生物学意义的代谢产物，迄今为止质谱分析难以对其进行表征，其快速原子轰击质谱图非常强烈，以母离子为主。高分辨率选择离子检测与恒定B/E比的联动扫描子离子分析相结合，能够明确鉴定患有丙酸血症、甲基丙二酸尿症和瑞氏综合征儿童尿液中的酰基肉碱。利用（2H3）乙酰肉碱和（2H5）丙酰肉碱作为内标，通过同位素稀释对选定样本中的乙酰肉碱和丙酰肉碱进行定量分析。这些技术将有助于评估左旋肉碱在这些疾病中的治疗用途。
