PROPIONYLCARNITINE AND FREE CARNITINE ARE NEW BIOMARKERS IN THE FOLLOW-UP PERIOD OF MUCOPOLYSACCARIDOSIS TO SCREEN OXIDATIVE STRESS

Abstract

Objective
 There is no applicable and easy procedure to screen
 oxidative stress in mucopolysaccaridosis (MPS)
 patients. The aim herein was to show the antioxidative
 properties of free carnitine (FC) and propionylcarnitine
 (PC) with respect to oxidative markers in MPS and
 use a simple and easy method in patient follow-up.
 Material and Methods
 FC and PC were studied as an antioxidative
 marker using tandem mass spectroscopy and
 malondialdehyde (MDA) was studied as an oxidative
 marker in 27 MPS patients and 24 healthy volunteers.
 Results
 While the PC and FC levels were significantly
 decreased, the MDA levels were higher in the
 MPS patients than in the healthy volunteers.When
 compared between the enzyme-treated MPS patients
 and untreated MPS patients, there were no significant
 differences between the groups. MDA was found to
 inversely correlated with PC (r =–0.402, P=0.003).It
 was noteworthy that PC had a significant correlation
 with MDA.
 Conclusion
 The findings revealed that the affected patients had
 higher MDA levels and lower PC and FC levels,
 indicating an imbalance through the oxidative side. An
 applicable method of FC and PC measurement could
 be used to screen patients, considering them as new
 antioxidative markers in the patient follow-up period
 for the response of enzyme replacement therapy and/
 or antioxidant drugs.