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Transcriptional downregulation of <jats:italic>fat<\/jats:italic> was previously observed in a <jats:italic>Drosophila<\/jats:italic> model of the polyglutamine disease Dentatorubral-pallidoluysian atrophy (DRPLA) and this was shown to be partially responsible for autophagy defects and neurodegeneration. However, it is still unclear whether a downregulation of mammalian <jats:italic>Fat<\/jats:italic> orthologues is associated with neurodegeneration in mice. We hereby show that all four <jats:italic>Fat<\/jats:italic> orthologues are transcriptionally downregulated in the cerebellum in a mouse model of DRPLA. To elucidate the possible roles of single <jats:italic>Fat<\/jats:italic> genes, this study concentrates on <jats:italic>Fat3<\/jats:italic>. This <jats:italic>fat<\/jats:italic> homologue is shown to be the most widely expressed in the brain. Conditional knockout (KO) of Fat3 in brains of adult mice was attempted using the inducible Thy1Cre(ER<jats:sup>T2<\/jats:sup>) SLICK H line. Behavioral and biochemical analysis revealed that mice with conditional KO of <jats:italic>Fat3<\/jats:italic> in the brain display no abnormalities. This may be ascribed either to the limited efficiency of the KO strategy pursued or to the lack of effect of <jats:italic>Fat3<\/jats:italic> KO on autophagy.<\/jats:p>","DOI":"10.1038\/s41598-019-52684-7","type":"journal-article","created":{"date-parts":[[2019,11,6]],"date-time":"2019-11-06T11:03:34Z","timestamp":1573038214000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Fat cadherins in mouse models of degenerative ataxias"],"prefix":"10.1038","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0536-8678","authenticated-orcid":false,"given":"Olga","family":"Baron","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Denise","family":"Grieshober","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Catarina","family":"Dias","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7807-2563","authenticated-orcid":false,"given":"Manolis","family":"Fanto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2019,11,6]]},"reference":[{"key":"52684_CR1","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1016\/j.ceb.2017.11.006","volume":"51","author":"S Blair","year":"2018","unstructured":"Blair, S. & McNeill, H. 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