Drosophila is a powerful model system to study human trinucleotide repeat diseases. Findings in Drosophila models highlighted importance of host proteins, chaperons, and protein clearance pathways in polyglutamine diseases as well as that of RNA-binding proteins in noncoding repeat RNA toxicity diseases. Recent novel aspects revealed in Drosophila models include pleiotropic Ataxin 2 interactions, antisense transcription in trinucleotide repeat diseases, contribution of CAG RNA in polyglutamine diseases, and the role of RNA foci in CUG expansion diseases. Drosophila models have been also used for repeat stability studies.
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