Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease.

@article{Gardian2005NeuroprotectiveEO,
  title={Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease.},
  author={Gabriella Gardian and Susan E. Browne and Dong-Kug Choi and P{\'e}ter Kliv{\'e}nyi and Jason Gregorio and James K. Kubilus and Hoon Ryu and Brett Langley and Rajiv R Ratan and Robert Ferrante and Myron Flint Beal},
  journal={The Journal of biological chemistry},
  year={2005},
  volume={280 1},
  pages={556-63}
}
Huntington's disease (HD) is caused by an expansion of exonic CAG triplet repeats in the gene encoding the huntingtin protein (Htt), however, the means by which neurodegeneration occurs remains obscure. There is evidence that mutant Htt interacts with transcription factors leading to reduced histone acetylation. We report that administration of the histone deacetylase inhibitor phenylbutyrate after onset of symptoms in a transgenic mouse model of HD significantly extends survival and attenuates… CONTINUE READING
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