Article
Ex vivo effect of JAK inhibition on JAK-STAT1 pathway hyperactivation in patients with dominant-negative STAT3 mutations.
Journal of clinical immunology - 1 Aug 2022
Lobo Pilar Blanco, Guisado-Hernández Paloma, Villaoslada Isabel, de Felipe Beatriz, Carreras Carmen, Rodriguez Hector, Carazo-Gallego Begoña, Méndez-Echevarria Ana, Lucena José Manuel, Aljaro Pilar Ortiz, Castro María José, Noguera-Uclés José Francisco, Milner Joshua D, McCann Katelyn, Zimmerman Ofer, Freeman Alexandra F, Lionakis Michail S, Holland Steven M, Neth Olaf, Olbrich Peter
Abstract excerpt
PURPOSE: STAT1 gain-of-function (GOF) and dominant-negative (DN) STAT3 syndromes share clinical manifestations including infectious and inflammatory manifestations. Targeted treatment with Janus-kinase (JAK) inhibitors shows promising results in treating STAT1 GOF-associated symptoms while management of DN STAT3 patients has been largely supportive. We here assessed the impact of ruxolitinib on the JAK-STAT1/3...
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