Transcriptional effects of CSB and the CSB-PGBD3 fusion protein in CSB-null UVSS1KO cells

ABSTRACT: Cockayne syndrome is a segmental progeria most often caused by mutations in the CSB gene encoding a SWI/SNF-like ATPase required for transcription-coupled DNA repair (TCR). Over 43 Mya before marmosets diverged from humans, a piggyBac3 (PGBD3) transposable element integrated into intron 5 of the CSB gene. As a result, primate CSB genes now generate both CSB protein and a conserved CSB-PGBD3 fusion protein in which the first 5 exons of CSB are alternatively spliced to the PGBD3 transposase. We show by microarray analysis that expression of the fusion protein alone in CSB-null UV-sensitive syndrome cells (UVSS1KO) cells induces an interferon-like response that resembles both the innate antiviral response and the prolonged interferon response normally maintained by unphosphorylated STAT1 (U-S

ORGANISM(S): Homo sapiens

SUBMITTER: Lucas Gray 

PROVIDER: E-GEOD-56049 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress