Çopur, O.Yazıcı, H.Canbay, E.Durmaz, B.Canda, E.Ucar, S.K.Coker, M.2024-08-252024-08-2520241043-4666https://doi.org/10.1016/j.cyto.2023.156410https://hdl.handle.net/11454/101050Recently, it has been shown disturbances in oxidant/antioxidant system and increases in some inflammatory markers in animal studies and in some Mucopolysaccharidoses (MPSs) patients. In this study, we aimed to determine the oxidative stress/antioxidant parameters and pro-inflammatory cytokine levels in the serum of MPS patients, in order to evaluate the possible role of inflammation in these patient groups regarding to accumulated metabolites. MPS I (n = 3), MPS II (n = 8), MPS III (n = 4), MPS IVA (n = 3), MPS VI (n = 3), and VII (n = 1) patients and 20 age-matched healthy subjects were included into the study. There was no statistically significant change in activities of SOD, Catalase, GSH-Px and lipid peroxidation levels in erythrocytes between the MPS patients and healthy controls. While IL-1alpha (p = 0.054), IL-6 (p = 0.008) levels, and chitotriosidase activity (p = 0.003) elevated in MPS3 patients, IL1? (p = 0.006), IL-1? (p = 0.006), IL-6 (p = 0.006), IFN? (p = 0.006), and NF?B (p = 0.006) levels increased in MPS-6 patients. Elevated levels of IL-6, IL1? and chitotriosidase activity demonstrated macrophage activation in MPSIII untreated with enzyme replacement. Our study showed for the first time that high levels of IL1?, IL-6, IL1? and NF?B were present in MPSVI patients, demonstrating the induction of inflammation by dermatan sulphate. The low level of paraoxonase in MPSVI patients may be a good marker for cardiac involvement. Overall, this study provides important insights into the relationship between lysosomal storage of glycosaminoglycan and inflammation in MPS patients. It highlights possible pathways for the increased release of inflammatory molecules and suggests new targets for the development of treatments. © 2023 Elsevier Ltden10.1016/j.cyto.2023.156410info:eu-repo/semantics/closedAccessInflammationMucopolysaccharidosisParaoxonasearyldialkylphosphatasecatalasechitotriosidasecytokinedermatan sulfategamma interferonglutathione peroxidaseglycosaminoglycanimmunoglobulin enhancer binding proteininterleukin 1alphainterleukin 1betainterleukin 6superoxide dismutaseadolescentadultantioxidant activityArticlechildclinical articlecontrolled studyenzyme deficiencyenzyme replacementerythrocytefemaleheart diseasehumanHunter syndromeHurler syndromeinflammationlipid peroxidationlysosome storage diseasemacrophage activationmaleMaroteaux Lamy syndromemetaboliteMorquio syndromemucopolysaccharidosismucopolysaccharidosis type 7oxidative stressSanfilippo syndromeArticle1732-s2.0-8517557719637924740Q2