Glycosaminoglycan-induced proinflammatory cytokine levels as disease marker in mucopolysaccharidosis

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dc.authorscopusid57936042000
dc.authorscopusid57202984757
dc.authorscopusid55896960200
dc.authorscopusid23472577300
dc.authorscopusid23059712400
dc.authorscopusid26428601400
dc.authorscopusid7007002166
dc.contributor.authorÇopur, O.
dc.contributor.authorYazıcı, H.
dc.contributor.authorCanbay, E.
dc.contributor.authorDurmaz, B.
dc.contributor.authorCanda, E.
dc.contributor.authorUcar, S.K.
dc.contributor.authorCoker, M.
dc.date.accessioned2024-08-25T18:38:31Z
dc.date.available2024-08-25T18:38:31Z
dc.date.issued2024
dc.departmentEge Üniversitesien_US
dc.description.abstractRecently, 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 Ltden_US
dc.identifier.doi10.1016/j.cyto.2023.156410
dc.identifier.issn1043-4666
dc.identifier.pmid37924740en_US
dc.identifier.scopus2-s2.0-85175577196en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.urihttps://doi.org/10.1016/j.cyto.2023.156410
dc.identifier.urihttps://hdl.handle.net/11454/101050
dc.identifier.volume173en_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherAcademic Pressen_US
dc.relation.ispartofCytokineen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.snmz20240825_Gen_US
dc.subjectInflammationen_US
dc.subjectMucopolysaccharidosisen_US
dc.subjectParaoxonaseen_US
dc.subjectaryldialkylphosphataseen_US
dc.subjectcatalaseen_US
dc.subjectchitotriosidaseen_US
dc.subjectcytokineen_US
dc.subjectdermatan sulfateen_US
dc.subjectgamma interferonen_US
dc.subjectglutathione peroxidaseen_US
dc.subjectglycosaminoglycanen_US
dc.subjectimmunoglobulin enhancer binding proteinen_US
dc.subjectinterleukin 1alphaen_US
dc.subjectinterleukin 1betaen_US
dc.subjectinterleukin 6en_US
dc.subjectsuperoxide dismutaseen_US
dc.subjectadolescenten_US
dc.subjectadulten_US
dc.subjectantioxidant activityen_US
dc.subjectArticleen_US
dc.subjectchilden_US
dc.subjectclinical articleen_US
dc.subjectcontrolled studyen_US
dc.subjectenzyme deficiencyen_US
dc.subjectenzyme replacementen_US
dc.subjecterythrocyteen_US
dc.subjectfemaleen_US
dc.subjectheart diseaseen_US
dc.subjecthumanen_US
dc.subjectHunter syndromeen_US
dc.subjectHurler syndromeen_US
dc.subjectinflammationen_US
dc.subjectlipid peroxidationen_US
dc.subjectlysosome storage diseaseen_US
dc.subjectmacrophage activationen_US
dc.subjectmaleen_US
dc.subjectMaroteaux Lamy syndromeen_US
dc.subjectmetaboliteen_US
dc.subjectMorquio syndromeen_US
dc.subjectmucopolysaccharidosisen_US
dc.subjectmucopolysaccharidosis type 7en_US
dc.subjectoxidative stressen_US
dc.subjectSanfilippo syndromeen_US
dc.titleGlycosaminoglycan-induced proinflammatory cytokine levels as disease marker in mucopolysaccharidosisen_US
dc.typeArticleen_US

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