Histone deacetylase inhibitors as therapeutics for polyglutamine disorders

R Butler, GP Bates - Nature Reviews Neuroscience, 2006 - nature.com
During the past 5 years, gene expression studies in cell culture, animal models and in the
brains of patients have shown that the perturbation of transcription frequently results in …

Histones associated with downregulated genes are hypo-acetylated in Huntington's disease models

G Sadri-Vakili, B Bouzou, CL Benn… - Human molecular …, 2007 - academic.oup.com
Transcriptional dysregulation plays a major role in the pathology of Huntington's disease
(HD). However, the mechanisms causing selective downregulation of genes remain …

Histone deacetylase activity is retained in primary neurons expressing mutant huntingtin protein

M Hoshino, K Tagawa, T Okuda… - Journal of …, 2003 - Wiley Online Library
Perturbation of histone acetyl‐transferase (HAT) activity is implicated in the pathology of
polyglutamine diseases, and suppression of the counteracting histone deacetylase (HDAC) …

Genomic landscape of transcriptional and epigenetic dysregulation in early onset polyglutamine disease

LM Valor, D Guiretti, JP Lopez-Atalaya… - Journal of …, 2013 - Soc Neuroscience
Transcriptional dysregulation is an important early feature of polyglutamine diseases. One of
its proposed causes is defective neuronal histone acetylation, but important aspects of this …

Mechanisms of disease: Histone modifications in Huntington's disease

G Sadri-Vakili, JHJ Cha - Nature Clinical Practice Neurology, 2006 - nature.com
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by
a polyglutamine repeat expansion within the huntingtin protein. HD is characterized by …

Histone deacetylase inhibitors reduce polyglutamine toxicity

A McCampbell, AA Taye, L Whitty… - Proceedings of the …, 2001 - National Acad Sciences
Polyglutamine diseases include at least nine neurodegenerative disorders, each caused by
a CAG repeat expansion in a different gene. Accumulation of mutant polyglutamine …

Histone deacetylase inhibitors: a novel therapeutic approach to Huntington's disease (complex mechanism of neuronal death).

G Sadri-Vakili, JHJ Cha - Current Alzheimer Research, 2006 - ingentaconnect.com
Huntington's disease is an autosomal dominantly inherited neurodegenerative disorder
caused by a polyglutamine repeat expansion. The onset of HD leads to problems with …

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

E Hockly, VM Richon, B Woodman… - Proceedings of the …, 2003 - National Acad Sciences
Huntington's disease (HD) is an inherited, progressive neurological disorder that is caused
by a CAG/polyglutamine repeat expansion and for which there is no effective therapy …

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

JS Steffan, L Bodai, J Pallos, M Poelman… - Nature, 2001 - nature.com
Proteins with expanded polyglutamine repeats cause Huntington's disease and other
neurodegenerative diseases. Transcriptional dysregulation and loss of function of …

Clinical features of Huntington's disease

R Ghosh, SJ Tabrizi - Polyglutamine disorders, 2018 - Springer
Huntington's disease (HD) is the most common monogenic neurodegenerative disease and
the commonest genetic dementia in the developed world. With autosomal dominant …