Abstract
More than 15 human genetic diseases, including Huntington's disease, result from the expansion of a trinucleotide repeat. The expansions are unstable in specific somatic tissues, which can lead to disease acceleration. Here we discuss the role of transcription elongation in tissue-selective trinucleotide repeat instability.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Cerebellum / metabolism
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Chromatin / chemistry
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Chromatin / metabolism
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Corpus Striatum / metabolism
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Genomic Instability
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Histones / metabolism
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Humans
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Huntingtin Protein
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Huntington Disease / genetics*
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Huntington Disease / metabolism
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Huntington Disease / pathology
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Mice
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Nerve Tissue Proteins / genetics
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Nerve Tissue Proteins / metabolism
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RNA Polymerase II / metabolism*
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RNA, Messenger / chemistry
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RNA, Messenger / metabolism
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Transcription Elongation, Genetic
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Trinucleotide Repeats
Substances
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Chromatin
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HTT protein, human
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Histones
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Huntingtin Protein
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Nerve Tissue Proteins
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RNA, Messenger
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RNA Polymerase II