{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T02:54:23Z","timestamp":1789786463338,"version":"4.0.1"},"reference-count":36,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2009,8,27]],"date-time":"2009-08-27T00:00:00Z","timestamp":1251331200000},"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":[[2009,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Bcl\u20102\u2010associated athanogene\u20101 (BAG1) is a multifunctional protein delivering chaperone\u2010recognized unfolded substrates to the proteasome for degradation. It has been shown to be essential for proper CNS development <jats:italic>in vivo,<\/jats:italic> playing a crucial role in neuronal survival and differentiation. With regard to Huntington\u2019s disease, a sequestration of BAG1 into inclusion bodies and a neuroprotective effect in double transgenic mice have been reported. Here, we show that BAG1 reduces aggregation and accelerates degradation of mutant huntingtin (htt\u2010mut). Moreover, it reduces nuclear levels of htt\u2010mut. This effect can be overcome by over\u2010expression of seven in absentia homolog 1, an E3 ligase negatively regulated by BAG1 and known to be involved in nuclear import of htt\u2010mut. <jats:italic>In vivo<\/jats:italic>, BAG1 proved to be protective in a <jats:italic>Drosophila\u00a0melanogaster<\/jats:italic> Huntington\u2019s disease model, preventing photoreceptor cell loss induced by htt\u2010mut. In summary, we present BAG1 as a therapeutic tool modulating key steps in htt toxicity <jats:italic>in vitro<\/jats:italic> and ameliorating htt toxicity <jats:italic>in vivo<\/jats:italic>.<\/jats:p>","DOI":"10.1111\/j.1471-4159.2009.06363.x","type":"journal-article","created":{"date-parts":[[2009,8,27]],"date-time":"2009-08-27T15:34:52Z","timestamp":1251387292000},"page":"801-807","source":"Crossref","is-referenced-by-count":31,"title":["BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution"],"prefix":"10.1111","volume":"111","author":[{"given":"Kamila","family":"Sroka","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aaron","family":"Voigt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Deeg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John 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