Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice
Beth Israel Deaconess Medical Center · Harvard University · +5 more institutions
Abstract
The precise cause of neuronal death in Huntington's disease (HD) is unknown. Although no single specific protein-protein interaction of mutant huntingtin has emerged as the pathologic trigger, transcriptional dysfunction may contribute to the neurodegeneration observed in HD. Pharmacological treatment using the histone deacetylase inhibitor sodium butyrate to modulate transcription significantly extended survival in a dose-dependent manner, improved body weight and motor performance, and delayed the neuropathological sequelae in the R6/2 transgenic mouse model of HD. Sodium butyrate also increased histone and Specificity protein-1 acetylation and protected against 3-nitropropionic acid neurotoxicity.…
Citation impact
- FWCI
- 13.03
- Percentile
- 100%
- References
- 72
Authors
11- RJRobert J. FerranteCorresponding
Beth Israel Deaconess Medical Center, Harvard University, Edith Nourse Rogers Memorial Veterans Hospital, Massachusetts General Hospital, Bedford VA Research Corporation, Geriatric Research Education and Clinical Center
- JKJames K. Kubilus
Boston University, Beth Israel Deaconess Medical Center, Harvard University, Edith Nourse Rogers Memorial Veterans Hospital, Massachusetts General Hospital, Geriatric Research Education and Clinical Center
- JLJunghee Lee
Boston University, Beth Israel Deaconess Medical Center, Harvard University, Edith Nourse Rogers Memorial Veterans Hospital, Massachusetts General Hospital, Geriatric Research Education and Clinical Center
- HRHoon Ryu
Boston University, Beth Israel Deaconess Medical Center, Harvard University, Edith Nourse Rogers Memorial Veterans Hospital, Massachusetts General Hospital, Geriatric Research Education and Clinical Center
- AAAyshe Ana Beesen
Boston University, Beth Israel Deaconess Medical Center, Harvard University, Edith Nourse Rogers Memorial Veterans Hospital, Massachusetts General Hospital, Geriatric Research Education and Clinical Center
Topics & keywords
- Sodium butyrate
- Histone deacetylase
- Phenotype
- Huntington's disease
- Cancer research
- Histone deacetylase inhibitor
- HDAC11
- Medicine
- Good health and well-being