Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Ipiranga 2752, Porto Alegre, RS, 90610-000, Brazil.
Clin Biochem. 2012 Jan;45(1-2):77-81. doi: 10.1016/j.clinbiochem.2011.10.017. Epub 2011 Nov 4.
We investigated the relationship between butyrylcholinesterase (BuChE) activity and lipid oxidative damage in patients with disorders of propionate metabolism, before and after treatment with protein restriction and L-carnitine.
BuChE activity and malondialdehyde (MDA) were measured in plasma from eight untreated patients (at diagnosis) and from seven patients under treatment with protein restriction and L-carnitne supplementation (100mg/kg/day).
We verified a significant reduction of butyrylcholinesterase activity, as well as an increased MDA formation in plasma from untreated patients. However, treated patients presented MDA and BuChE activity similar to controls. Furthermore, butyrylcholinesterase activity was negatively correlated with MDA concentrations in these patients.
The results suggest that an increased free radicals formation may be involved in the decrease of butyrylcholinesterase activity, possibly contributing to the neurological damage of these disorders, and that treatment with L-carnitine and low-protein diet possibly is able to prevent this damage.
我们研究了丙酸代谢紊乱患者在接受蛋白质限制和左旋肉碱治疗前后，丁酰胆碱酯酶（BuChE）活性与脂质氧化损伤之间的关系。
测量了 8 例未经治疗患者（诊断时）和 7 例接受蛋白质限制和左旋肉碱补充治疗（100mg/kg/天）患者的血浆 BuChE 活性和丙二醛（MDA）。
我们发现未经治疗的患者的 BuChE 活性显著降低，同时血浆 MDA 生成增加。然而，经过治疗的患者的 MDA 和 BuChE 活性与对照组相似。此外，这些患者的 BuChE 活性与 MDA 浓度呈负相关。
结果表明，自由基的形成增加可能与 BuChE 活性的降低有关，这可能导致这些疾病的神经损伤，而左旋肉碱和低蛋白饮食的治疗可能能够预防这种损伤。
