Nucleotide excision repair, mismatch repair, and R-loops modulate convergent transcription-induced cell death and repeat instability

• Published in: PloS one Vol. 7; no. 10; p. e46807
• Main Authors: Lin, Yunfu, Wilson, John H
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 03.10.2012; Public Library of Science (PLoS)
• Subjects: Antisense; Antisense RNA; Apoptosis; Ataxia; Biochemistry; Biology; Blotting, Western; Cell cycle; Cell death; Cellular stress response; Convergence; Deoxyribonucleic acid; Depletion; Development and progression; Disease; DNA; DNA damage; DNA Mismatch Repair - genetics; DNA repair; DNA Repair - genetics; DNA-Binding Proteins - genetics; DNA-Binding Proteins - metabolism; Drosophila; Genes; Genomes; Genomic Instability - genetics; Human behavior; Human tissues; Humans; Hybrids; Insects; Instability; Medicine; Mismatch repair; Modulators; Molecular biology; Mortality; MSH2 protein; Nervous system diseases; Neurodegenerative diseases; Neuromodulation; Neurons; Nucleotide excision repair; Polyglutamine; R-loops; Recognition; Repair; Ribonuclease; Ribonuclease H1; Ribonucleic acid; RNA; RNA, Small Interfering; Rodents; siRNA; Structural stability; Studies; Tissues; Transcription; Transcription (Genetics); Transcription, Genetic - genetics; Trinucleotide repeat diseases; Trinucleotide repeats; Texas

Expansion of CAG•CTG tracts located in specific genes is responsible for 13 human neurodegenerative disorders, the pathogenic mechanisms of which are not yet well defined. These disease genes are ubiquitously expressed in human tissues, and transcription has been identified as one of the major pathw...