Journal
ANTIOXIDANTS
Volume 10, Issue 3, Pages -Publisher
MDPI
DOI: 10.3390/antiox10030357
Keywords
brain; cystatin; EPM1; neuro inflammation; LPS-induced oxidative stress; peroxiredoxin; thioredoxin; thioredoxin reductase
Funding
- Slovenian Research Agency [P-0140]
- Commissariat a l'EnergieAtomique et aux EnergiesAlternatives (CEA) France-Slovenia grant
- COST Action Evolution-Age-Gender-Lifestyle-Environment: mitochondrial fitnessmapping (MitoEAGLE) [CA15203]
- COST action An Integrative Action forMultidisciplinary Studies on Cellular Structural Networks (EuroCellNet) [CA15214]
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The study investigated the impact of lipopolysaccharide-triggered neuroinflammation on the protein levels of redox-sensitive proteins in the brain and cerebella of Stefin B-deficient mice. The results suggest a role of oxidative stress in the progression of the disease.
Stefin B (cystatin B) is an inhibitor of endo-lysosomal cysteine cathepsin, and the loss-of-function mutations in the stefin B gene were reported in patients with Unverricht-Lundborg disease (EPM1), a form of progressive myoclonus epilepsy. Stefin B-deficient mice, a mouse model of the disease, display key features of EPM1, including myoclonic seizures. Although the underlying mechanism is not yet completely clear, it was reported that the impaired redox homeostasis and inflammation in the brain contribute to the progression of the disease. In the present study, we investigated if lipopolysaccharide (LPS)-triggered neuroinflammation affected the protein levels of redox-sensitive proteins: thioredoxin (Trx1), thioredoxin reductase (TrxR), peroxiredoxins (Prxs) in brain and cerebella of stefin B-deficient mice. LPS challenge was found to result in a marked elevation of Trx1 and TrxR in the brain and cerebella of stefin B deficient mice, while Prx1 was upregulated only in cerebella after LPS challenge. Mitochondrial peroxiredoxin 3 (Prx3), was upregulated also in the cerebellar tissue lysates prepared from unchallenged stefin B deficient mice, while after LPS challenge Prx3 was upregulated in stefin B deficient brain and cerebella. Our results imply the role of oxidative stress in the progression of the disease.
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