{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Wang J"],"funding":["NIMH NIH HHS","NIGMS NIH HHS"],"pagination":["535-43"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC171394"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["112(4)"],"pubmed_abstract":["The type I IFNs (IFN-alpha and IFN-beta), which are crucial in antiviral defense and immune regulation, signal via the Janus kinase/signal transducer and activator of transcription (JAK/STAT) pathway with activation of STAT1 and STAT2. Here, the function of STAT2 was studied in transgenic mice (termed GIFN/STAT2-/-) with CNS production of IFN-alpha. Surprisingly, GIFN/STAT2-/-, but not GIFN/STAT1-null, transgenic mice, with CNS production of IFN-alpha, died prematurely with medulloblastoma. An immune response also induced in the brain of the GIFN/STAT2-/- mice was associated with IFN-gamma gene expression by CD3+ T cells and the activation of the STAT1, STAT3, STAT4, and STAT5 molecules. Expression of the Sonic hedgehog (Shh) and the downstream transcriptional factor Gli-1 genes, implicate"],"journal":["The Journal of clinical investigation"],"pubmed_title":["Dysregulated Sonic hedgehog signaling and medulloblastoma consequent to IFN-alpha-stimulated STAT2-independent production of IFN-gamma in the brain."],"pmcid":["PMC171394"],"funding_grant_id":["P30 MH062261","R01 MH062231","MH-62231","MH-62261","GM-54686"],"pubmed_authors":["Pham-Mitchell N","Campbell IL","Wang J","Schindler C"],"additional_accession":[]},"is_claimable":false,"name":"Dysregulated Sonic hedgehog signaling and medulloblastoma consequent to IFN-alpha-stimulated STAT2-independent production of IFN-gamma in the brain.","description":"The type I IFNs (IFN-alpha and IFN-beta), which are crucial in antiviral defense and immune regulation, signal via the Janus kinase/signal transducer and activator of transcription (JAK/STAT) pathway with activation of STAT1 and STAT2. Here, the function of STAT2 was studied in transgenic mice (termed GIFN/STAT2-/-) with CNS production of IFN-alpha. Surprisingly, GIFN/STAT2-/-, but not GIFN/STAT1-null, transgenic mice, with CNS production of IFN-alpha, died prematurely with medulloblastoma. An immune response also induced in the brain of the GIFN/STAT2-/- mice was associated with IFN-gamma gene expression by CD3+ T cells and the activation of the STAT1, STAT3, STAT4, and STAT5 molecules. Expression of the Sonic hedgehog (Shh) and the downstream transcriptional factor Gli-1 genes, implicate","dates":{"release":"2003-01-01T00:00:00Z","publication":"2003 Aug","modification":"2025-04-04T19:07:04.665Z","creation":"2019-03-27T00:35:25Z"},"accession":"S-EPMC171394","cross_references":{"pubmed":["12925694"],"doi":["10.1172/JCI200318637","10.1172/JCI18637","10.1172/jci200318637"]}}