The conserved Cockayne syndrome B-piggyBac fusion protein (CSB-PGBD3) affects DNA repair and induces both interferon-like and innate antiviral responses in CSB-null cells

► Cockayne syndrome is a devasting childhood progeria most often caused by defects in the CSB gene. ► The CSB gene encodes a repair and chromatin remodeling SWI/SNF ATPase. ► The CSB gene also generates an abundant CSB-PGBD3 fusion protein that joins the N-terminus of CSB to a piggyBac transposase. ► The fusion protein affects DNA repair, and induces an interferon-like response in CSB-null cells. ► We speculate regarding the function of the conserved fusion protein in health and disease.