geranylgeranylacetone and Job-Syndrome

Autosomal dominant hyper-IgE syndrome (AD-HIES) is caused by dominant-negative mutations in STAT3; however, the molecular basis for mutant STAT3 allele dysfunction is unclear and treatment remains supportive. We hypothesized that AD-HIES mutations decrease STAT3 protein stability and that mutant STAT3 activity can be improved by agents that increase chaperone protein activity. We used computer modeling to characterize the effect of STAT3 mutations on protein stability. We measured STAT3 protein half-life (t

Topics: Animals; CD4-Positive T-Lymphocytes; CD8-Positive T-Lymphocytes; Cytokines; Diterpenes; DNA-Binding Proteins; Half-Life; Heat Shock Transcription Factors; Herpesvirus 4, Human; Humans; Interleukin-17; Job Syndrome; Mice; Models, Molecular; Mutant Proteins; Mutation; Phosphotyrosine; Protein Binding; Protein Stability; Spleen; STAT3 Transcription Factor; Transcription Factors