Matches in SemOpenAlex for { <https://semopenalex.org/work/W4382361750> ?p ?o ?g. }
- W4382361750 abstract "Individuals with Down syndrome (DS) display chronic hyperactivation of interferon signaling. However, the clinical impacts of interferon hyperactivity in DS are ill-defined. Here, we describe a multiomics investigation of interferon signaling in hundreds of individuals with DS. Using interferon scores derived from the whole blood transcriptome, we defined the proteomic, immune, metabolic, and clinical features associated with interferon hyperactivity in DS. Interferon hyperactivity associates with a distinct proinflammatory phenotype and dysregulation of major growth signaling and morphogenic pathways. Individuals with the highest interferon activity display the strongest remodeling of the peripheral immune system, including increased cytotoxic T cells, B cell depletion, and monocyte activation. Interferon hyperactivity accompanies key metabolic changes, most prominently dysregulated tryptophan catabolism. High interferon signaling stratifies a subpopulation with elevated rates of congenital heart disease and autoimmunity. Last, a longitudinal case study demonstrated that JAK inhibition normalizes interferon signatures with therapeutic benefit in DS. Together, these results justify the testing of immune-modulatory therapies in DS." @default.
- W4382361750 created "2023-06-29" @default.
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- W4382361750 date "2023-06-28" @default.
- W4382361750 modified "2023-10-14" @default.
- W4382361750 title "Multidimensional definition of the interferonopathy of Down syndrome and its response to JAK inhibition" @default.
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- W4382361750 doi "https://doi.org/10.1126/sciadv.adg6218" @default.
- W4382361750 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/37379383" @default.
- W4382361750 hasPublicationYear "2023" @default.