AI Article Synopsis
- L-carnitine plays a crucial role in transporting fatty acids and protecting cells from oxidative damage, and its levels were investigated in PKU patients adhering to a strict diet.
- The study found that patients strictly following their low-protein diet had significantly lower serum L-carnitine levels compared to controls, while those not adhering to the diet showed higher levels.
- Additionally, oxidative stress markers indicated increased damage in PKU patients, leading researchers to suggest that monitoring L-carnitine levels in these patients could prompt potential supplementation as part of their treatment.
Article Abstract
Aims: L-carnitine exerts an important role by facilitating the mitochondrial transport of fatty acids, but is also a scavenger of free radicals, protecting cells from oxidative damage. Phenylketonuria (PKU), an inborn error of phenylalanine (Phe) metabolism, is currently treated with a special diet consisting of severe restriction of protein-enriched foods, therefore potentially leading to L-carnitine depletion. The aim of this study was to determine L-carnitine levels and oxidative stress parameters in blood of two groups of PKU patients, with good and poor adherence to treatment.
Methods: Treatment of patients consisted of a low protein diet supplemented with a synthetic amino acids formula not containing Phe, L-carnitine, and selenium. L-carnitine concentrations and the oxidative stress parameters thiobarbituric acid reactive species (TBARS) and total antioxidant reactivity (TAR) were measured in blood of the two groups of treated PKU patients and controls.
Results: We verified a significant decrease of serum L-carnitine levels in patients who strictly adhered to the diet, as compared to controls and patients who did not comply with the diet. Furthermore, TBARS measurement was significantly increased and TAR was significantly reduced in both groups of phenylketonuric patients relatively to controls. We also found a significant negative correlation between TBARS and L-carnitine levels and a significant positive correlation between TAR and L-carnitine levels in well-treated PKU patients.
Conclusions: Our results suggest that L-carnitine should be measured in plasma of treated PKU patients, and when a decrease of this endogenous component is detected in plasma, supplementation should be considered as an adjuvant therapy.
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| http://dx.doi.org/10.1007/s10571-008-9313-y | DOI Listing |
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