- Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotectiveZhihua Qiu
Massachusetts General Institute for Neurodegenerative Disease, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, 114 16th Street, Charlestown, MA 02129, USA
J Biol Chem 281:16672-80. 2006..Our data suggest that enhancement of transcription factor Sp1 contributes to the pathology of HD and demonstrates that its suppression is beneficial...
- Transcriptional dysregulation in striatal projection- and interneurons in a mouse model of Huntington's disease: neuronal selectivity and potential neuroprotective role of HAP1Birgit Zucker
Department of Neurology, MassGeneral Institute for Neurodegenerative Diseases, Massachusetts General Hospital and Harvard Medical School, Boston 02129, USA
Hum Mol Genet 14:179-89. 2005....
- Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregationJonathan H Fox
MassGeneral Institute for Neurodegenerative Disease, Department of Neurology, Massachusetts General Hospital, Charlestown, Massachusetts, USA
J Neurochem 91:413-22. 2004..We suggest that cystamine's neuroprotective effect in HD transgenic mice results from pleiotropic effects that include transglutaminase inhibition and antioxidant activity...
- Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease miceRobert J Ferrante
Geriatric Research Education and Clinical Center, Bedford Veterans Affairs Medical Center, Bedford, Massachusetts 01730, USA
J Neurosci 23:9418-27. 2003....
- Gabapentin-lactam, but not gabapentin, reduces protein aggregates and improves motor performance in a transgenic mouse model of Huntington's diseaseBirgit Zucker
Department of Neurology, University Hospital Freiburg, Breisacherstrasse 64, 79106 Freiburg, Germany
Naunyn Schmiedebergs Arch Pharmacol 370:131-9. 2004..The pharmacokinetics of GBP-L yielded a mean plasma concentration near the EC50 of GBP-L to open mitochondrial ATP-dependent K+ channels. These findings support the role of GBP-L as a novel neuroprotective substance in vivo...