Neurobiology of Disease Widespread Disruption of Repressor Element-1 Silencing Transcription Factor/Neuron-Restrictive Silencer Factor Occupancy at Its Target Genes in Huntington’s Disease
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چکیده
Chiara Zuccato,1* Nikolai Belyaev,2* Paola Conforti,1 Lezanne Ooi,2 Marzia Tartari,1 Evangelia Papadimou,1 Marcy MacDonald,3 Elisa Fossale,3 Scott Zeitlin,4 Noel Buckley,5 and Elena Cattaneo1 1Department of Pharmacological Sciences and Centre for Stem Cell Research, University of Milan, Via Balzaretti 9, 20133 Milano, Italy, 2Institute of Membrane and Systems Biology, University of Leeds, Leeds LS2 9JT, United Kingdom, 3Center for Human Genetic Research, Massachusetts General Hospital Richard B. Simches Research Center, Boston, Massachusetts 02114, 4Department of Neuroscience, University of Virginia School of Medicine, Charlottesville, Virginia 22908, and 5Centre for the Cellular Basis of Behaviour, The James Black Centre, Institute of Psychiatry, King’s College London, London SE5 9NU, United Kingdom
منابع مشابه
Widespread disruption of repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy at its target genes in Huntington's disease.
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 trans...
متن کاملLoss of huntingtin function complemented by small molecules acting as repressor element 1/neuron restrictive silencer element silencer modulators.
Increased levels of the repressor element 1/neuron restrictive silencer element (RE1/NRSE) silencing activity promoter, and a consequent reduction in the transcription of many RE1/NRSE-bearing neuronal genes, including brain-derived neurotrophic factor (BDNF), have been demonstrated in Huntington disease (HD) and represent one possible effector of its selective neuronal vulnerability. Restoring...
متن کاملNon-Coding RNAs in Neural Networks, REST-Assured
In the nervous system, several key steps in cellular complexity and development are regulated by non-coding RNAs (ncRNAs) and the repressor element-1 silencing transcription factor/neuron-restrictive silencing factor (REST/NRSF). REST recruits gene regulatory complexes to regulatory sequences, among them the repressor element-1/neuron-restrictive silencer element, and mediates developmental sta...
متن کاملConstitutive expression of the neuron-restrictive silencer factor (NRSF)/REST in differentiating neurons disrupts neuronal gene expression and causes axon pathfinding errors in vivo.
The neuron-restrictive silencer factor (NRSF; also known as REST for repressor element-1 silencing transcription factor) is a transcriptional repressor of multiple neuronal genes, but little is known about its function in vivo. NRSF is normally down-regulated upon neuronal differentiation. Constitutive expression of NRSF in the developing spinal cord of chicken embryos caused repression of two ...
متن کاملGenome-wide analysis of repressor element 1 silencing transcription factor/neuron-restrictive silencing factor (REST/NRSF) target genes.
The completion of whole genome sequencing projects has provided the genetic instructions of life. However, whereas the identification of gene coding regions has progressed, the mapping of transcriptional regulatory motifs has moved more slowly. To understand how distinct expression profiles can be established and maintained, a greater understanding of these sequences and their trans-acting fact...
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تاریخ انتشار 2007