作者
Alexandre Kuhn, Darlene R Goldstein, Angela Hodges, Andrew D Strand, Thierry Sengstag, Charles Kooperberg, Kristina Becanovic, Mahmoud A Pouladi, Kirupa Sathasivam, Jang-Ho J Cha, Anthony J Hannan, Michael R Hayden, Blair R Leavitt, Stephen B Dunnett, Robert J Ferrante, Roger Albin, Peggy Shelbourne, Mauro Delorenzi, Sarah J Augood, Richard LM Faull, James M Olson, Gillian P Bates, Lesley Jones, Ruth Luthi-Carter
发表日期
2007/8/1
期刊
Human molecular genetics
卷号
16
期号
15
页码范围
1845-1861
出版商
Oxford University Press
简介
To test the hypotheses that mutant huntingtin protein length and wild-type huntingtin dosage have important effects on disease-related transcriptional dysfunction, we compared the changes in mRNA in seven genetic mouse models of Huntington's disease (HD) and postmortem human HD caudate. Transgenic models expressing short N-terminal fragments of mutant huntingtin (R6/1 and R6/2 mice) exhibited the most rapid effects on gene expression, consistent with previous studies. Although changes in the brains of knock-in and full-length transgenic models of HD took longer to appear, 15- and 22-month CHL2Q150/Q150, 18-month HdhQ92/Q92 and 2-year-old YAC128 animals also exhibited significant HD-like mRNA signatures. Whereas it was expected that the expression of full-length huntingtin transprotein might result in unique gene expression changes compared with those caused by the expression of …
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A Kuhn, DR Goldstein, A Hodges, AD Strand… - Human molecular genetics, 2007