Ceccarelli M, Cortigiani L, Assanta N, Nutini P, Ughi C
Istituto di Clinica Pediatrica, Università degli Studi di Pisa.
Minerva Pediatr. 1992 Sep;44(9):401-5.
Carnitine is a very important co-factor for the metabolism of fatty acids, because it is the necessary carrier for the passage of acyl groups inside the mitochondria, where beta-oxidation takes place. In the human body the total pool of carnitine is made by two fractions, one being endogenous and the second exogenous. The absorption of exogenous carnitine takes place mainly at duodenal-jejunal level by an active transport mechanism based on amino acid carrier. Plasma L-carnitine concentrations have been measured in 66 coeliac patients (mean age: 7 years and 4 months). Thirty three of them were on a free diet and the other 33 were on a gluten-free diet for at last six month. In 10 patients we studied plasma L-carnitine levels both on a free diet and on a gluten-free diet. As controls we examined 33 healthy children comparable for age. Plasma L-carnitine concentrations have been measured by a spectrophotometrical method according to Marquis and Fritz's technique and subsequently modified by Pearson and Seccombe. In the 66 coeliac patients the mean values of serum L-carnitine were significantly lower than those in the controls (p < 0.001). The levels were significantly lower in patients a free diet with respect to those on gluten-free diet (p < 0.01). The 10 subjects who were examined both on free diet and on gluten-free diet showed an increase of plasma concentrations in the latter condition.(ABSTRACT TRUNCATED AT 250 WORDS)
肉碱是脂肪酸代谢中非常重要的辅助因子，因为它是酰基在线粒体内传递的必要载体，而β-氧化作用发生在线粒体内。在人体中，肉碱的总储备由两部分组成，一部分是内源性的，另一部分是外源性的。外源性肉碱的吸收主要发生在十二指肠-空肠水平，通过基于氨基酸载体的主动转运机制进行。我们测量了66例乳糜泻患者（平均年龄：7岁4个月）的血浆左旋肉碱浓度。其中33例患者采用自由饮食，另外33例患者至少六个月来一直采用无麸质饮食。我们对10例患者在自由饮食和无麸质饮食两种情况下的血浆左旋肉碱水平都进行了研究。作为对照，我们检查了33名年龄相仿的健康儿童。血浆左旋肉碱浓度采用根据马奎斯和弗里茨技术并随后经皮尔逊和西科姆修改的分光光度法进行测量。在66例乳糜泻患者中，血清左旋肉碱的平均值显著低于对照组（p<0.001）。采用自由饮食的患者的水平显著低于采用无麸质饮食的患者（p<0.01）。对10名在自由饮食和无麸质饮食两种情况下都接受检查的受试者而言，后一种情况下血浆浓度有所升高。（摘要截选至250词）
