Analysis of the repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy of non-neuronal genes in peripheral lymphocytes from patients with Huntington's disease.

Brain Pathology
Manuela MarulloChiara Zuccato

Abstract

We have previously demonstrated that the transcription of neuronal repressor element-1/neuron-restrictive silencer element (RE1/NRSE)-regulated genes is reduced in the brain of subjects with Huntington's disease (HD) as a result of increased binding of the repressor element-1 silencing transcription factor/neuron-restrictive silencer factor (REST/NRSF) to its RE1/NRSE targets. As specific non-neuronal REST/NRSF-regulated genes have been identified in the human genome, we exploited the possibility that the binding of REST/NRSF to its target RE1/NRSE sites may also be altered in the peripheral tissues of HD patients. Our results show that REST/NRSF occupancy is increased in lymphocytes from HD subjects, thus indicating for the first time that the activity of the RE1/NRSE sites is dysfunctional in vivo. Chromatin immunoprecipitation (ChIP) of the RE1/NRSE sites in lymphocytes may therefore be a reproducible, sensitive and specific means of searching for candidate markers of HD onset and progression.

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Citations

Jun 15, 2011·Current Neurology and Neuroscience Reports·Irfan A Qureshi, Mark F Mehler
Oct 23, 2013·Trends in Molecular Medicine·Irfan A Qureshi, Mark F Mehler
Apr 13, 2010·Brain Research·Irfan A QureshiMark F Mehler
Mar 19, 2014·Neuropathology and Applied Neurobiology·Davide SchifferChiara Zuccato
Dec 11, 2013·Bioinformatics and Biology Insights·Jun-Ichi SatohYoji Yamamoto
Feb 11, 2016·Movement Disorders Clinical Practice·Johannes K EhingerEskil Elmér
Jun 6, 2009·Nature Reviews. Neurology·Chiara Zuccato, Elena Cattaneo
Jan 21, 2021·Molecular Oncology·Shavali ShaikVidya Gopalakrishnan

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