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- Title
An expanded role for wild-type huntingtin in neuronal transcription.
- Authors
Thompson, Leslie Michels
- Abstract
The article highlights an important role for the normal protein in neuronal transcriptional regulation. Huntington disease is caused by a polyglutamine repeat expansion in the huntingtin protein. Repressor element-1 transcription factor/neuron restrictive silencer factor (REST/NRSF) was one of the first negatively acting transcriptional regulators found to be involved in vertebrate neuronal development. A gene such as that encoding the m4 muscarinic acetylcholine receptor may be expressed in postmitotic neurons of the cortex but not in liver cell. Huntington disease is one of nine triplet repeat diseases caused by a CAG repeat expansion in the coding region of a given gene9. Huntingtin is expressed in nearly all tissues in the body, but because levels of REST/NRSF are high in non-neuronal tissues, transcription of NRSF-regulated genes will potentially be repressed even in the absence of wild-type huntingtin.
- Subjects
GENETIC transcription; PROTEINS; HUNTINGTON disease; NEURAL circuitry; ACETYLCHOLINE
- Publication
Nature Genetics, 2003, Vol 35, Issue 1, p13
- ISSN
1061-4036
- Publication type
Article
- DOI
10.1038/ng0903-13