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.

@article{Kuhn2007MutantHE,
  title={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.},
  author={Alexandre Kuhn and Darlene R. Goldstein and Angela K. Hodges and Andrew D. Strand and Thierry Sengstag and Charles Kooperberg and Kristina Be{\vc}anovi{\'c} and Mahmoud A Pouladi and Kirupa Sathasivam and J J Cha and Anthony J Hannan and Michael R. Hayden and Blair R. Leavitt and Stephen B. Dunnett and Robert Ferrante and Roger Albin and Peggy Shelbourne and Mauro Delorenzi and Sarah J. Augood and Richard L. M. Faull and James M. Olson and Gillian P Bates and Lesley Jones and Ruth Luthi-Carter},
  journal={Human molecular genetics},
  year={2007},
  volume={16 15},
  pages={1845-61}
}
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… CONTINUE READING
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Modulation of nuleosome dynamics in Huntington’s disease

  • E. C. Stack, S. J. Del Signore, +8 authors S.W
  • Hum. Mol. Genet,
  • 2007
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