Inflammatory profile in X-linked adrenoleukodystrophy patients: Understanding disease progression
| Autor: | Desirèe Padilha Marchetti, Patricia Koehler-Santos, Bruna Donida, Adriana Simon Coitinho, Carmen Regla Vargas, Laura Bannach Jardim, Daniella de Moura Coelho, Tatiane Cristina Hauschild, Marion Deon, Carlos Eduardo Diaz Jacques |
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| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Adult Male endocrine system Adolescent medicine.medical_treatment Interleukin-1beta Inflammation Brain damage Biochemistry Asymptomatic 03 medical and health sciences Young Adult 0302 clinical medicine Adrenal insufficiency Medicine Humans Adrenoleukodystrophy Child Molecular Biology business.industry Tumor Necrosis Factor-alpha Macrophages Fatty Acids Infant Cell Biology Middle Aged Th1 Cells medicine.disease Pathophysiology Interleukin-10 030104 developmental biology Cytokine Child Preschool Immunology Biomarker (medicine) Cytokines Interleukin-2 Interleukin-4 medicine.symptom Interleukin-5 business 030217 neurology & neurosurgery |
| Zdroj: | Journal of cellular biochemistry. 119(1) |
| ISSN: | 1097-4644 |
| Popis: | X-linked adrenoleukodystrophy (X-ALD) is an inherited disease characterized by progressive inflammatory demyelization in the brain, adrenal insufficiency, and an abnormal accumulation of very long chain fatty acids (VLCFA) in tissue and body fluids. Considering that inflammation might be involved in pathophysiology of X-ALD, we aimed to investigate pro- and anti-inflammatory cytokines in plasma from three different male phenotypes (CCER, AMN, and asymptomatic individuals). Our results showed that asymptomatic patients presented increased levels of pro-inflammatory cytokines IL-1β, IL-2, IL-8, and TNF-α and the last one was also higher in AMN phenotype. Besides, asymptomatic patients presented higher levels of anti-inflammatory cytokines IL-4 and IL-10. AMN patients presented higher levels of IL-2, IL-5, and IL-4. We might hypothesize that inflammation in X-ALD is related to plasmatic VLCFA concentration, since there were positive correlations between C26:0 plasmatic levels and pro-inflammatory cytokines in asymptomatic and AMN patients and negative correlation between anti-inflammatory cytokine and C24:0/C22:0 ratio in AMN patients. The present work yields experimental evidence that there is an inflammatory imbalance associated Th1, (IL-2, IL-6, and IFN-γ), Th2 (IL-4 and IL-10), and macrophages response (TNF-α and IL-1β) in the periphery of asymptomatic and AMN patients, and there is correlation between VLCFA plasmatic levels and inflammatory mediators in X-ALD. Furthermore, we might also speculate that the increase of plasmatic cytokines in asymptomatic patients could be considered an early biomarker of brain damage and maybe also a predictor of disease progression. |
| Databáze: | OpenAIRE |
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