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Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.
Kuhn A, Goldstein DR, Hodges A, Strand AD, Sengstag T, Kooperberg C, Becanovic K, Pouladi MA, Sathasivam K, Cha JH, Hannan AJ, Hayden MR, Leavitt BR, Dunnett SB, Ferrante RJ, Albin R, Shelbourne P, Delorenzi M, Augood SJ, Faull RL, Olson JM, Bates GP, Jones L, Luthi-Carter R. Kuhn A, et al. Hum Mol Genet. 2007 Aug 1;16(15):1845-61. doi: 10.1093/hmg/ddm133. Epub 2007 May 21. Hum Mol Genet. 2007. PMID: 17519223
SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis.
Luthi-Carter R, Taylor DM, Pallos J, Lambert E, Amore A, Parker A, Moffitt H, Smith DL, Runne H, Gokce O, Kuhn A, Xiang Z, Maxwell MM, Reeves SA, Bates GP, Neri C, Thompson LM, Marsh JL, Kazantsev AG. Luthi-Carter R, et al. Proc Natl Acad Sci U S A. 2010 Apr 27;107(17):7927-32. doi: 10.1073/pnas.1002924107. Epub 2010 Apr 8. Proc Natl Acad Sci U S A. 2010. PMID: 20378838 Free PMC article.
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