Protein stabilization improves STAT3 function in autosomal dominant hyper-IgE syndrome
Bocchini C, Nahmod K, Katsonis P, Kim S, Kasembeli M, Freeman A, Lichtarge O, Makedonas G, Tweardy D. Protein stabilization improves STAT3 function in autosomal dominant hyper-IgE syndrome. Blood 2016, 128: 3061-3072. PMID: 27799162, PMCID: PMC5201093, DOI: 10.1182/blood-2016-02-702373.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCD4-Positive T-LymphocytesCD8-Positive T-LymphocytesCytokinesDiterpenesDNA-Binding ProteinsHalf-LifeHeat Shock Transcription FactorsHerpesvirus 4, HumanHumansInterleukin-17Job SyndromeMiceModels, MolecularMutant ProteinsMutationPhosphotyrosineProtein BindingProtein StabilitySpleenSTAT3 Transcription FactorTranscription FactorsConceptsAutosomal dominant hyper-IgE syndromeSTAT3 mutationsPeripheral blood mononuclear cellsAD-HIESProtein stabilityEpstein-Barr virus-transformed BPY-STAT3Human peripheral blood mononuclear cellsHeat shock proteinsHyper-IgE syndromeSTAT3 target genesProtein half-lifeMouse splenocytesUpregulating heat shock proteinsAD-HIES patientsLevel of STAT3T cellsDominant-negative mutationsTreatment of human peripheral blood mononuclear cellsCD8<sup>+</sup> T cellsSTAT3 functionEpstein-Barr virus-transformed B cellsProtein functionHuman CD4<sup>+</sup>MRNA levels