Burns S P, Iles R A, Saudubray J M, Chalmers R A
Medical Unit, London Hospital Medical College, UK.
Eur J Pediatr. 1996 Jan;155(1):31-5. doi: 10.1007/BF02115623.
Propionylcarnitine (PC) excretion has been measured during a clinical trial of metronidazole therapy in two patients with propionic acidaemia and two patients with methylmalonic aciduria. All patients were in good metabolic control and were receiving L-carnitine. While total propionate excretion was reduced by up to 40% in all four patients during metronidazole therapy, the excretion of propionylcarnitine remained largely unchanged. PC comprised up to 80% of total propionate excretion in patients with propionic acidaemia.
These results suggest an extra-hepatic source and/or differing compartmentation for PC formation from those for the production of other metabolites of propionyl-CoA.
在一项甲硝唑治疗的临床试验中，对两名丙酸血症患者和两名甲基丙二酸尿症患者的丙酰肉碱（PC）排泄情况进行了测量。所有患者代谢控制良好，且正在接受左旋肉碱治疗。在甲硝唑治疗期间，所有四名患者的总丙酸排泄量最多减少了40%，但丙酰肉碱的排泄量基本保持不变。在丙酸血症患者中，PC占总丙酸排泄量的比例高达80%。
这些结果表明，与丙酰辅酶A其他代谢产物的产生相比，PC形成的来源在肝外和/或存在不同的区室化。
