{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T10:38:01Z","timestamp":1775644681023,"version":"3.50.1"},"reference-count":50,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2021,2,27]],"date-time":"2021-02-27T00:00:00Z","timestamp":1614384000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Antioxidants"],"abstract":"<jats:p>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\u2013Lundborg 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.<\/jats:p>","DOI":"10.3390\/antiox10030357","type":"journal-article","created":{"date-parts":[[2021,2,27]],"date-time":"2021-02-27T20:55:44Z","timestamp":1614459344000},"page":"357","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Altered Expression of Peroxiredoxins in Mouse Model of Progressive Myoclonus Epilepsy upon LPS-Induced Neuroinflammation"],"prefix":"10.3390","volume":"10","author":[{"given":"Mojca","family":"Trstenjak Prebanda","sequence":"first","affiliation":[{"name":"Department of Biochemistry, Molecular and Structural Biology, Jo\u017eef Stefan Institute, SI-1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petra","family":"Matjan-\u0160tefin","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Molecular and Structural Biology, Jo\u017eef Stefan Institute, SI-1000 Ljubljana, Slovenia"},{"name":"International Postgraduate School Jo\u017eef Stefan, Jamova 39, SI-1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9007-5764","authenticated-orcid":false,"given":"Boris","family":"Turk","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Molecular and Structural Biology, Jo\u017eef Stefan Institute, SI-1000 Ljubljana, Slovenia"},{"name":"Faculty of Chemistry and Chemical Technology, University of Ljubljana, Ve\u010dna pot 113, SI-1000 Ljubljana, Slovenia"},{"name":"Institute of Regenerative Medicine, I.M. Sechenov First Moscow State Medical University, Bol\u2019shaya Pirogovskaya Ulitsa, 19c1, 119146 Moscow, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1381-070X","authenticated-orcid":false,"given":"Nata\u0161a","family":"Kopitar-Jerala","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Molecular and Structural Biology, Jo\u017eef Stefan Institute, SI-1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1111\/j.1528-1167.2008.01546.x","article-title":"Clinical picture of EPM1-Unverricht-Lundborg disease","volume":"49","author":"Khyuppenen","year":"2008","journal-title":"Epilepsia"},{"key":"ref_2","first-page":"342","article-title":"Identification of mutations in cystatin B, the gene responsible for the Unverricht-Lundborg type of progressive myoclonus epilepsy (EPM1)","volume":"60","author":"Lalioti","year":"1997","journal-title":"Am. 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