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Journals / Medicine Science / 2021 / Cilt: 10 - Sayı: 4

Do cytokines play role in the pathogenesis of mucopolysaccharidosis

Gursel Biberoglu
Asli Inci
Ilyas Okur
Leyla Tumer
Fatih Suheyl Ezgu
Canan Demirtas
Pages
1492–1497
Publication Date
2022-10-18
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Although enzyme replacement therapy reduces glycosaminoglycan accumulation by preventing cytokine production, it might be more effective when given with antiinfllammatory mediators.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:The mucopolysaccharidoses (MPS) are a heterogeneous group of inborn errors of metabolism with an increased deposition of glycosaminoglycans in lysosomes (GAGs).
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Key Findings & Data:Forty-three MPS patients as patient group and 29 healthy children as control group were included in the study.
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Conclusion & Impact:Although enzyme replacement therapy reduces glycosaminoglycan accumulation by preventing cytokine production, it might be more effective when given with antiinfllammatory mediators.
🔬Design:Modeling & Simulation
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

The mucopolysaccharidoses (MPS) are a heterogeneous group of inborn errors of metabolism with an increased deposition of glycosaminoglycans in lysosomes (GAGs). In MPS, GAG leads to inflammatory pathway activation. In MPS models, studies have shown that accumulation of glycosaminoglycans in lysosomes causes activation of oxidative stress, and then apoptosis is triggered. In this study, the aim was to show whether GAG deposition could trigger the inflammatory processes via cytokines. Forty-three MPS patients as patient group and 29 healthy children as control group were included in the study. Samples were taken before and after ERT from 8 MPS patients. 15 patients were treated with enzyme replacement and 28 patients could not be treated with the enzyme. Tumor necrosis alpha (TNF-α), interleukin 1-beta (IL-1β), interleukin 6 (IL-6) levels were studied by ELISA method. When cytokine levels of MPS patients and control groups were compared, cytokines were found to significantly increased (p≤0.05) in MPS patients. Pretreatment IL-6 and posttreatment IL-6 and IL-1β levels of patients treated with enzyme replacement therapy (ERT) were found near to control group. Pre-treatment and post-treatment TNF-α levels were found significantly higher in MPS patients than the control group that was significantly higher in the post-treatment group. In the MPS group of ERT treatment, IL-1β and TNF-α levels decreased significantly after ERT(p≤0.05). MPS patients had higher levels of IL-6 and TNF-α levels than the control group that revealed inflammatory pathway activation in MPS patients. The inflammatory process is prominent in MPS patients that GAG deposition leads to increase pro-inflammatory cytokines and oxidative stress. Although enzyme replacement therapy reduces glycosaminoglycan accumulation by preventing cytokine production, it might be more effective when given with antiinfllammatory mediators.

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Cite This Article

Biberoglu, G., Inci, A., Okur, I., Tumer, L., Ezgu, F. S., Demirtas, C. (2022). Do cytokines play role in the pathogenesis of mucopolysaccharidosis. Medicine Science, Cilt: 10 - Sayı: 4, 1492–1497. https://ajindex.com/articles/579700

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References (13)

  1. Wraith JE. The mucopolysaccharidoses: a clinical review and guide to management. Arch Dis Child,1995;72:263–7
  2. Muenzer J, Wraith JE, L Clarke A. Mucopolysaccharidosis I: management and treatment guidelines. Pediatrics 2009;123:19–29
  3. Muenzer J. The mucopolysaccharidoses: a heterogeneous group of disorders with variable pediatric presentations. J Pediatr 2004;144:27–34
  4. Ohmi K, Greenberg DS, Rajavel KS, et al. Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB. Proc Natl Acad Sci USA 2003;100:1902–7
  5. Bernsen PL, Wevers RA, Gabreels FJ, et al. Phenotypic expression in mucopolysaccharidosis VII. J Neurol Neurosurg Psychiatry 1987;50:699– 703
  6. Cleary MA, Wraith JE. Management of mucopolysaccharidosis type III. Arch Dis Child 1993; 69:403–6
  7. Langford-Smith A, Langford-Smith KJ, Jones SA, et al. Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field test. PLoS One 201;6:25717 8. Langford-Smith A, Malinowska M, Langford-Smith KJ, et al.Hyperactive behaviour in the mouse model of mucopolysaccharidosis IIIB in the open field and home cage environments. Genes Brain Behav, 2011;10:673–82 9. Vitner EB, Platt EM,Futerman AH. Common and uncommon pathogenic cascades in lysosomal storage diseases. J Biol Chem 2010;285:20423–7
  8. Simonaro CM, Haskins ME, Schuchman EH. Articular chondrocytes from animals with a dermatan sulfate storage disease undergo a high rate of apoptosis and release nitric oxide and inflammatory cytokines: A possible mechanism underlying degenerative joint disease in the mucopolysaccharidoses. Lab Invest 2001;81:1319–28
  9. Simonaro CM, D’Angelo M, Haskins ME, et al. Joint and bone disease in mucopolysaccharidoses VI and VII: Identification of new therapeutic targets and biomarkers using animal models. Pediatr Res 2005; 57:701–7
  10. Simonaro CM, D'Angelo M, He X, et al. Mechanism of glycosaminoglycanmediated bone and joint disease: Implications for the mucopolysaccharidoses and other connective tissue diseases. Am J Pathol 2008;172:112–22
  11. Hoshino K. Cutting edge: Toll-like receptor 4 (TLR4)-deficient mice are hyporesponsive to lipopolysaccharide: Evidence for TLR4 as the Lps gene product. J Immunol,1999;162:3749–52
  12. Taylor KR, Yamasaki K, Radek KA, et al. Recognition of hyaluronan released in sterile injury involves a unique receptor complex dependent on Toll-like receptor 4, CD44, and MD-2. J Biol Chem 2007;282:18265–75
  13. Xia Y,