Chemically Induced Increases and Decreases in the Rate of Expansion of a CAG centre dot CTG Triplet Repeat;Mutagenic Stress Modulates the Dynamics of CTG Repeat Instability Associated With Myotonic Dystrophy Type 1;Replication Inhibitors Modulate Ins

摘要

The genomic instability of CTG/CAG trinucleotide repeats is the hallmark of at least 14 diseases,including myotonic dystrophy type 1(DM1). During the last few years,research has attempted to decipher the mechanisms involved in the particularly intriguing dynamics of CTG/CAG repeats. The identification of chemical and environmental factors capable of modifying the rate of repeat expansion presents an interesting challenge. Such findings could possibly provide important clues about the mechanisms of repeat size mutation and open promising routes toward therapy. This review highlights three recent articles that describe the possibility of chemical modulation of CTG repeat instability in mammalian cell systems derived from DM1 patients or transgenic mouse models.