Artuch R, Colomé C, Vilaseca M A, Sierra C, Cambra F J, Lambruschini N, Campistol J
Servei de Bioquímica, Hospital Sant Joan de Déu/Hospital Clinic, Universitat de Barcelona, Spain.
J Inherit Metab Dis. 2001 Jun;24(3):359-66. doi: 10.1023/a:1010500502275.
Decreased serum ubiquinone-10 concentrations is a common condition in patients with phenylketonuria (PKU) under dietary treatment. Our aim was to investigate the implication of the metabolic abnormalities of PKU (low concentrations of tyrosine and high concentrations of phenylalanine) and the effect of treatment with phenylalanine-restricted diets in decreased ubiquinone-10 concentrations in PKU patients. We studied 30 PKU patients (age range 5 months to 35 years; median age 7 years) under dietary treatment. Correlation between plasma tyrosine or phenylalanine and serum ubiquinone-10 concentrations was investigated. Daily cholesterol intake was calculated from the data obtained through a dietary questionnaire. The index of dietary control (IDC) was calculated as the average of the medians of plasma phenylalanine concentrations obtained every 6 months in the metabolic control of patients. Negative correlations were observed between serum ubiquinone and the IDC (r=-0.46; p<0.01) in PKU patients. No correlation was observed between tyrosine or daily cholesterol intake and serum ubiquinone concentrations. After adjustment for daily cholesterol intake by multiple linear regression analysis, for each 113 units of increase in IDC values serum ubiquinone decreased 0.1 micromol/L. According to our results, the main factor associated with the decreased serum ubiquinone concentrations was high plasma phenylalanine values. Although daily cholesterol intake seems to be associated with ubiquinone concentrations, it may not be relevant if we take into account the low intake of cholesterol in treated PKU patients.
血清辅酶Q10浓度降低是接受饮食治疗的苯丙酮尿症（PKU）患者的常见情况。我们的目的是研究PKU代谢异常（酪氨酸浓度低和苯丙氨酸浓度高）的影响以及采用低苯丙氨酸饮食治疗对PKU患者辅酶Q10浓度降低的作用。我们研究了30例接受饮食治疗的PKU患者（年龄范围5个月至35岁；中位年龄7岁）。研究了血浆酪氨酸或苯丙氨酸与血清辅酶Q10浓度之间的相关性。通过饮食调查问卷获得的数据计算每日胆固醇摄入量。饮食控制指数（IDC）计算为患者代谢控制中每6个月获得的血浆苯丙氨酸浓度中位数的平均值。在PKU患者中，观察到血清辅酶Q与IDC之间呈负相关（r = -0.46；p <0.01）。未观察到酪氨酸或每日胆固醇摄入量与血清辅酶Q浓度之间的相关性。通过多元线性回归分析对每日胆固醇摄入量进行校正后，IDC值每增加113个单位，血清辅酶Q降低0.1微摩尔/升。根据我们的结果，与血清辅酶Q浓度降低相关的主要因素是血浆苯丙氨酸值高。虽然每日胆固醇摄入量似乎与辅酶Q浓度有关，但如果考虑到接受治疗的PKU患者胆固醇摄入量低，可能就不相关了。
