A hexamer tandem repeat RNA embedded within an SVA retrotransposon drives R-loop formation and neurodegeneration
D’Ignazio et al. developed ventral forebrain organoids to model the caudate nucleus of X-linked dystonia parkinsonism patients. A thorough characterization led to the identification of SVA retroelement-containing repeat transcripts associated with R-loops and neurodegeneration. The study offers clues on how targeting repeat transcripts using antisense oligonucleotides could rescue neurodegeneration.
