{"owner": "ArrayExpress Uploader", "pop_total": 0, "species": "human", "factors": [{"GSM1354423": {"SELECTION": "hygromycin (FLAG-HA-CSB) and neomycin (FLAG-CSB-PGBD3)", "GENOTYPE": "CSB_CSB-PGBD3"}}, {"GSM1354423": {"SELECTION": "hygromycin (FLAG-HA-CSB) and neomycin (FLAG-CSB-PGBD3)", "GENOTYPE": "CSB_CSB-PGBD3"}}, {"GSM1354423": {"SELECTION": "hygromycin (FLAG-HA-CSB) and neomycin (FLAG-CSB-PGBD3)", "GENOTYPE": "CSB_CSB-PGBD3"}}, {"GSM1354420": {"SELECTION": "hygromycin", "GENOTYPE": "CSB-PGBD3"}}, {"GSM1354420": {"SELECTION": "hygromycin", "GENOTYPE": "CSB-PGBD3"}}, {"GSM1354420": {"SELECTION": "hygromycin", "GENOTYPE": "CSB-PGBD3"}}, {"GSM1354417": {"SELECTION": "hygromycin", "GENOTYPE": "CSB"}}, {"GSM1354417": {"SELECTION": "hygromycin", "GENOTYPE": "CSB"}}, {"GSM1354417": {"SELECTION": "hygromycin", "GENOTYPE": "CSB"}}, {"GSM1354414": {"SELECTION": "hygromycin", "GENOTYPE": "control"}}, {"GSM1354414": {"SELECTION": "hygromycin", "GENOTYPE": "control"}}, {"GSM1354414": {"SELECTION": "hygromycin", "GENOTYPE": "control"}}], "id": 2096, "ownerprofile_id": "arrayexpress_sid", "platform": 4, "summary_wrapped": "Cockayne syndrome is a segmental progeria most often caused by mutations in the CSB gene encoding a SWI/SNF-like ATPase required for...", "pubmed_id": 22483866, "geo_gse_id": "E-GEOD-56049", "owner_profile": "/profile/8773/arrayexpressuploader", "factor_count": 2, "sample_count": 12, "tags": ["cockayne syndrome", "disease", "insulin", "intron", "progeria", "protein", "syndrome"], "lastmodified": "Dec.12, 2014", "is_default": false, "geo_id_plat": "E-GEOD-56049_A-AFFY-44", "slug": "transcriptional-effects-of-csb-and-the-csb-pgbd3-f", "geo_gds_id": "", "name": "Transcriptional effects of CSB and the CSB-PGBD3 fusion protein in CSB-null UVSS1KO cells", "created": "Jul.11, 2014", "summary": "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-STAT1); moreover, as might be expected based on conservation of the fusion protein, this potentially cytotoxic interferon-like response is largely reversed by coexpression of functional CSB protein. Interestingly, expression of CSB and the CSB-PGBD3 fusion protein together, but neither alone, upregulates the insulin growth factor binding protein IGFBP5 and downregulates IGFBP7, suggesting that the fusion protein may also confer a metabolic advantage, perhaps in the presence of DNA damage. Finally, we show that the fusion protein binds in vitro to members of a dispersed family of 900 internally deleted piggyBac elements known as MER85s, providing a potential mechanism by which the fusion protein could exert widespread effects on gene expression. Our data suggest that the CSB-PGBD3 fusion protein is important in both health and disease, and could play a role in Cockayne syndrome. 12 samples total; 4 gene expression conditions in triplicate; 1 condition is a tag-only negative control", "source": "http://www.ebi.ac.uk/arrayexpress/experiments/E-GEOD-56049", "sample_source": "http://www.ebi.ac.uk/arrayexpress/experiments/E-GEOD-56049/samples/"}