{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T01:50:02Z","timestamp":1791078602689,"version":"4.1.0"},"reference-count":50,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2012,3,28]],"date-time":"2012-03-28T00:00:00Z","timestamp":1332892800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2012,5]]},"abstract":"<jats:sec><jats:label\/><jats:p><jats:italic>J. Neurochem.<\/jats:italic> (2012) <jats:bold>121<\/jats:bold>, 639\u2013648.<\/jats:p><\/jats:sec><jats:sec><jats:title>Abstract<\/jats:title><jats:p>The transcription factor Elk\u20101 has been revealed as neuroprotective against toxic stimuli. In this study, we explored the neuroprotective capacity of Elk\u20101 in Huntington\u2019s disease. To this aim, we used two exon\u20101 mutant huntingtin (mhtt) mouse models (R6\/1 and R6\/2), and a full\u2010length mhtt striatal cell model (STHdh<jats:sup>Q111\/Q111<\/jats:sup>). Analysis of Elk\u20101 and pElk\u20101<jats:sup>Ser383<\/jats:sup> in the striatum of R6 mice revealed increased levels during the disease progression. Similarly, Elk\u20101 and pElk\u20101<jats:sup>Ser383<\/jats:sup> levels were increased in STHdh<jats:sup>Q111\/Q111<\/jats:sup> cells when compared with wild\u2010type cells. In addition, we observed a predominant nuclear localization of Elk\u20101 in STHdh<jats:sup>Q111\/Q111<\/jats:sup> cells, and in the striatum of 30\u2010week\u2010old R6\/1 mice. Nuclear Elk\u20101 did not colocalize with mhtt aggregates, suggesting a higher transcriptional activity. In agreement, the knock\u2010down of Elk\u20101 decreased immediate early genes expression in STHdh<jats:sup>Q111\/Q111<\/jats:sup> cells, but not in wild\u2010type cells. Interestingly, reduction of Elk\u20101 levels by siRNAs transfection promoted cell death and caspase 3 cleavage in STHdh<jats:sup>Q111\/Q111<\/jats:sup> cells, but not in wild\u2010type cells. In summary, we propose that increased protein levels, phosphorylation and nuclear localization of Elk\u20101 observed in exon\u20101 and full\u2010length Huntington\u2019s disease models could be a compensatory mechanism activated by striatal cells in response to the presence of mhtt that contributes to neuroprotection.<\/jats:p><\/jats:sec>","DOI":"10.1111\/j.1471-4159.2012.07711.x","type":"journal-article","created":{"date-parts":[[2012,2,28]],"date-time":"2012-02-28T18:02:33Z","timestamp":1330452153000},"page":"639-648","source":"Crossref","is-referenced-by-count":28,"title":["Activation of Elk\u20101 participates as a neuroprotective compensatory mechanism in models of Huntington\u2019s 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