+ Site Statistics
References:
54,258,434
Abstracts:
29,560,870
PMIDs:
28,072,757
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Oxidative and nitrative stress and pro-inflammatory cytokines in Mucopolysaccharidosis type II patients: effect of long-term enzyme replacement therapy and relation with glycosaminoglycan accumulation



Oxidative and nitrative stress and pro-inflammatory cytokines in Mucopolysaccharidosis type II patients: effect of long-term enzyme replacement therapy and relation with glycosaminoglycan accumulation



Biochimica et Biophysica Acta 1862(9): 1608-1616



Mucopolysaccharidosis type II (MPS II) is a lysosomal storage disease caused by a deficient activity of iduronate-2-sulfatase, leading to abnormal accumulation of glycosaminoglycans (GAG). The main treatment for MPS II is enzyme replacement therapy (ERT). Previous studies described potential benefits of six months of ERT against oxidative stress in patients. Thus, the aim of this study was to investigate oxidative, nitrative and inflammatory biomarkers in MPS II patients submitted to long term ERT. It were analyzed urine and blood samples from patients on ERT (mean time: 5.2years) and healthy controls. Patients presented increased levels of lipid peroxidation, assessed by urinary 15-F2t-isoprostane and plasmatic thiobarbituric acid-reactive substances. Concerning to protein damage, urinary di-tyrosine (di-Tyr) was increased in patients; however, sulfhydryl and carbonyl groups in plasma were not altered. It were also verified increased levels of urinary nitrate+nitrite and plasmatic nitric oxide (NO) in MPS II patients. Pro-inflammatory cytokines IL-1β and TNF-α were increased in treated patients. GAG levels were correlated to di-Tyr and nitrate+nitrite. Furthermore, IL-1β was positively correlated with TNF-α and NO. Contrastingly, we did not observed alterations in erythrocyte superoxide dismutase, catalase, glutathione peroxidase and glutathione reductase activities, in reduced glutathione content and in the plasmatic antioxidant capacity. Although some parameters were still altered in MPS II patients, these results may suggest a protective role of long-term ERT against oxidative stress, especially upon oxidative damage to protein and enzymatic and non-enzymatic defenses. Moreover, the redox imbalance observed in treated patients seems to be GAG- and pro-inflammatory cytokine-related.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 058488675

Download citation: RISBibTeXText

PMID: 27251652

DOI: 10.1016/j.bbadis.2016.05.021


Related references

Oxidative stress in patients with mucopolysaccharidosis type II before and during enzyme replacement therapy. Molecular Genetics and Metabolism 103(2): 121-127, 2011

Oxidative stress and inflammation in mucopolysaccharidosis type IVA patients treated with enzyme replacement therapy. Biochimica et Biophysica Acta 1852(5): 1012-1019, 2015

Mutational and oxidative stress analysis in patients with mucopolysaccharidosis type I undergoing enzyme replacement therapy. Clinica Chimica Acta; International Journal of Clinical Chemistry 387(1-2): 75-79, 2007

Bull's eye maculopathy and subfoveal deposition in two mucopolysaccharidosis type I patients on long-term enzyme replacement therapy. American Journal of Ophthalmology Case Reports 9: 1-6, 2018

Biomarker responses correlate with antibody status in mucopolysaccharidosis type I patients on long-term enzyme replacement therapy. Molecular Genetics and Metabolism 114(2): 129-137, 2015

Enzyme replacement therapy with double-dose Iaronidase every other week is safe and effective: case reports and glycosaminoglycan excretion patterns in ten mucopolysaccharidosis type I patients. Molecular Genetics and Metabolism 111(2): S101-S102, 2014

Long-term enzyme replacement therapy in a severe case of mucopolysaccharidosis type II (Hunter syndrome). European Review for Medical and Pharmacological Sciences 15(3): 253-258, 2011

Long-term effects of enzyme replacement therapy for Taiwanese patients with mucopolysaccharidosis IVA. Pediatrics and Neonatology 2018, 2018

Immune tolerance after long-term enzyme-replacement therapy among patients who have mucopolysaccharidosis I. Lancet 361(9369): 1608-1613, 2003

Immune tolerance in mucopolysaccharidosis I patients after long term enzyme replacement therapy. Journal of Inherited Metabolic Disease 26(Supplement 2): 140, 2003

Murine mucopolysaccharidosis type VII: long term therapeutic effects of enzyme replacement and enzyme replacement followed by bone marrow transplantation. Journal of Clinical Investigation 99(7): 1596-1605, 1997

Long-term galsulfase enzyme replacement therapy in Taiwanese mucopolysaccharidosis VI patients: A case series. Molecular Genetics and Metabolism Reports 7: 63-69, 2016

Effect of rosiglitazone on inflammatory cytokines and oxidative stress after intensive insulin therapy in patients with newly diagnosed type 2 diabetes. Diabetology and Metabolic Syndrome 11: 35, 2019

Early versus late treatment of spinal cord compression with long-term intrathecal enzyme replacement therapy in canine mucopolysaccharidosis type I. Molecular Genetics and Metabolism 101(2-3): 115-122, 2011

Enzyme replacement therapy for mucopolysaccharidosis VI: evaluation of long-term pulmonary function in patients treated with recombinant human N-acetylgalactosamine 4-sulfatase. Journal of Inherited Metabolic Disease 33(1): 51-60, 2010