Article
Widespread disruption of repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy at its target genes in Huntington's disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 27 Jun 2007
Zuccato Chiara, Belyaev Nikolai, Conforti Paola, Ooi Lezanne, Tartari Marzia, Papadimou Evangelia, MacDonald Marcy, Fossale Elisa, Zeitlin Scott, Buckley Noel, Cattaneo Elena
Abstract excerpt
Huntingtin is a protein that is mutated in Huntington's disease (HD), a dominant inherited neurodegenerative disorder. We previously proposed that, in addition to the gained toxic activity of the mutant protein, selective molecular dysfunctions in HD may represent the consequences of the loss of wild-type protein activity. We first reported that wild-type huntingtin positively affects the transcription of the...
Topics
- Animals
- Brain Chemistry
- Brain-Derived Neurotrophic Factor
- Cell Line, Transformed
- Corpus Striatum
- Down-Regulation
- Gene Expression Regulation
- Genetic Predisposition to Disease
