Oxidative damage and redox in Lysosomal Storage Disorders: Biochemical markers
| Autor: | Bruna Donida, Carlos Eduardo Diaz Jacques, Caroline Paula Mescka, Desirèe Padilha Marchetti, Graziela S. Ribas, Carmen Regla Vargas, Daiane Rodrigues, Roberto Giugliani |
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| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Antioxidant medicine.medical_treatment Clinical Biochemistry Cell Context (language use) Oxidative phosphorylation medicine.disease_cause Biochemistry Antioxidants 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Lysosome medicine Humans Chemistry Biochemistry (medical) PPT1 General Medicine Glutathione Lysosomal Storage Diseases Oxidative Stress 030104 developmental biology medicine.anatomical_structure Oxidation-Reduction Biomarkers 030217 neurology & neurosurgery Oxidative stress |
| Zdroj: | Clinica Chimica Acta. 466:46-53 |
| ISSN: | 0009-8981 |
| Popis: | Lysosomal Storage Disorders (LSD) comprise a heterogeneous group of >50 genetic disorders caused by mutations in genes that encode lysosomal enzymes, transport proteins or other gene products essential for a functional lysosomal system. As a result, abnormal accumulation of substrates within the lysosome leads to a progressive cellular impairment and dysfunction of numerous organs and systems. The exact mechanisms underlying the pathophysiology of LSD remain obscure. Previous studies proposed a relationship between oxidative stress and the pathogenesis of several inborn errors of metabolism, including LSD. Considering these points, in this paper it was reviewed oxidative stress and emerging antioxidant therapy in LSD, emphasizing studies with biological samples from patients affected by this group of conditions. These studies allow presuming that metabolites accumulated in LSD cause an increase of lysosomes' number and size, which may induce excessive production of reactive species and/or deplete the tissue antioxidant capacity, leading to damage in biomolecules. In vitro and in vivo evidence showed that cell oxidative process occurs in LSD and probably contributes to the pathophysiology of these disorders. In this context, it is possible to suggest that, in the future, antioxidants could come to be used as adjuvant therapy for LSD patients. |
| Databáze: | OpenAIRE |
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