{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T17:44:16Z","timestamp":1783446256791,"version":"3.55.0"},"reference-count":69,"publisher":"Public Library of Science (PLoS)","issue":"12","license":[{"start":{"date-parts":[[2022,12,27]],"date-time":"2022-12-27T00:00:00Z","timestamp":1672099200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Campagneteam Huntington"},{"name":"Campagneteam Huntington"},{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:p>Huntington\u2019s disease is an autosomal dominant heritable disorder caused by an expanded CAG trinucleotide repeat at the N-terminus of the <jats:italic>Huntingtin<\/jats:italic> (<jats:italic>HTT<\/jats:italic>) gene. Lowering the levels of soluble mutant HTT protein prior to aggregation through increased degradation by the proteasome would be a therapeutic strategy to prevent or delay the onset of disease. Native PAGE experiments in <jats:italic>Hdh<\/jats:italic>Q150 mice and R6\/2 mice showed that PA28\u03b1\u03b2 disassembles from the 20S proteasome during disease progression in the affected cortex, striatum and hippocampus but not in cerebellum and brainstem. Modulating PA28\u03b1\u03b2 activated proteasomes in various <jats:italic>in vitro<\/jats:italic> models showed that PA28\u03b1\u03b2 improved polyQ degradation, but decreased the turnover of mutant HTT. Silencing of PA28\u03b1\u03b2 in cells lead to an increase in mutant HTT aggregates, suggesting that PA28\u03b1\u03b2 is critical for overall proteostasis, but only indirectly affects mutant HTT aggregation.<\/jats:p>","DOI":"10.1371\/journal.pone.0278130","type":"journal-article","created":{"date-parts":[[2022,12,27]],"date-time":"2022-12-27T18:32:28Z","timestamp":1672165948000},"page":"e0278130","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":7,"title":["Reduction in PA28\u03b1\u03b2 activation in HD mouse brain correlates to increased mHTT aggregation in cell models"],"prefix":"10.1371","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2828-3378","authenticated-orcid":true,"given":"Karlijne W.","family":"Geijtenbeek","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jolien","family":"Janzen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleksandra E.","family":"Bury","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia","family":"Sanz-Sanz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ron A.","family":"Hoebe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie K.","family":"Bondulich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gillian P.","family":"Bates","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric A. 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