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We report that the tumor suppressor Homologous to the E6-AP Carboxyl Terminus domain and Ankyrin repeat containing E3 ubiquitin\u2013protein ligase 1 (HACE1) promotes activity of the transcription factor, nuclear factor erythroid 2-related factor 2 (NRF2), a master regulator of the antioxidative stress response. In Huntington disease patients, HACE1 is lost in the brain region most affected by the disease, namely the striatum, and restoring HACE1 functions in striatal cells expressing mutant Huntingtin protein provides protection against oxidative stress. Therefore, the tumor suppressor HACE1 is a new regulator of NRF2 and an emerging player in neurodegeneration.<\/jats:p>","DOI":"10.1073\/pnas.1314421111","type":"journal-article","created":{"date-parts":[[2014,2,11]],"date-time":"2014-02-11T01:09:20Z","timestamp":1392080960000},"page":"3032-3037","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":110,"title":["HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response"],"prefix":"10.1073","volume":"111","author":[{"given":"Barak","family":"Rotblat","sequence":"first","affiliation":[{"name":"Department of Molecular Oncology, British Columbia Cancer Research Centre, Vancouver, BC, Canada V5Z 1L3;"},{"name":"Medical Research Council, Toxicology Unit, Leicester University, Leicester LE1 9HN, United Kingdom;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amber 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