Analyses of oxidative stress biomarkers in Mucopolysaccharidosis VI compared with Mucopolysaccharidosis I and healthy controls

Abstract

High levels of reactive oxygen species have been associated with oxidative stress and inflammation and are related to changes in cellular characteristics caused by altered metabolic pathways of individuals with inborn errors of metabolism. Mucopolysaccharidosis (MPS) are characterized by biomolecular and tissue damage that results in the accumulation of undegraded glycosaminoglycans in cells of various organs and systems. The aim of this study was to measure superoxide dismutase (SOD), catalase (CAT) and thiobarbituric acid reactive substances (TBARS) in the plasma of individuals with MPS VI and compare these parameters with those of individuals with MPS I and healthy controls. The biomarkers of oxidative stress were evaluated in the plasma of individuals with MPS I and MPS VI and of healthy controls. The antioxidant capacity of blood plasma was measured by assessing SOD and CAT enzyme contents. The SOD results were 0.88±0.77 USOD/mg protein in the control group 0.89±0.56 USOD/mg protein in MPS I and 0.71±0.49 USOD/mg protein in MPS VI. The CAT activity in the control group was 83.24±36.24 UCAT/mg protein, 17.30±13.98 UCAT/mg protein in MPS I, and 36.79±19.30 UCAT protein in MPS VI. The TBARS analysis results were 1.23±0.52nmol/mg protein for the control group, 3.86±1.52nmol/mg protein for MPS I, and 3.13±1.19mol/mg de protein for MPS VI. In conclusion the results show that SOD enzyme activity did not change in either MPS, however, CAT activity decreased and a larger amount of TBARS was observed, which suggests both types of MPS are susceptible to oxidative damage.