title: Tissue-dependent regulation of RNAP II dynamics: the missing link between transcription and trinucleotide repeat instability in diseases?
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.
authors.affiliation: Programme of Translational Medicine and Neurogenetics; Institute of Genetics and Molecular and Cellular Biology (IGBMC); UMR 7104-CNRS/INSERM/UdS; Illkirch, France.
authors.forename: Agathi-Vasiliki
authors.initials: AV
authors.lastname: Goula
authors.forename: Richard
authors.initials: R
authors.lastname: Festenstein
authors.forename: Karine
authors.initials: K
authors.lastname: Merienne
mesh_terms: D000818:Animals; D002531:Cerebellum / Q000378:metabolism; D002843:Chromatin / Q000737:chemistry / Q000378:metabolism; D003342:Corpus Striatum / Q000378:metabolism; D042822:Genomic Instability; D006657:Histones / Q000378:metabolism; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D051379:Mice; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism; D012319:RNA Polymerase II / Q000378:metabolism*; D012333:RNA, Messenger / Q000737:chemistry / Q000378:metabolism; D061805:Transcription Elongation, Genetic; D018911:Trinucleotide Repeats
chemical_list: D002843:Chromatin; C086055:HTT protein, human; D006657:Histones; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D012333:RNA, Messenger; D012319:RNA Polymerase II