Diminished allergic disease in patients with STAT3 mutations reveals a role for STAT3 signaling in mast cell degranulation

• Published in: Journal of allergy and clinical immunology Vol. 132; no. 6; pp. 1388 - 1396.e3
• Main Authors: Siegel, Andrea M., Stone, Kelly D., Cruse, Glenn, Lawrence, Monica G., Olivera, Ana, Jung, Mi-yeon, Barber, John S., Freeman, Alexandra F., Holland, Steven M., O'Brien, Michelle, Jones, Nina, Wisch, Laura B., Kong, Heidi H., Desai, Avanti, Farber, Orly, Gilfillan, Alasdair M., Rivera, Juan, Milner, Joshua D.
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01.12.2013; Elsevier; Elsevier Limited
• Subjects: Adolescent; Adult; Aged; Allergies; Allergy and Immunology; anaphylaxis; Animals; atopic dermatitis; Autosomal dominant hyper-IgE syndrome; Biological and medical sciences; blood serum; Cell Degranulation - genetics; Cells, Cultured; Child; Child, Preschool; crosslinking; degranulation; Dermatitis; Dermatitis, Atopic - genetics; Dermatitis, Atopic - immunology; Disease; Female; Food; Food allergies; Food Hypersensitivity - epidemiology; Food Hypersensitivity - genetics; Food Hypersensitivity - immunology; Fundamental and applied biological sciences. Psychology; Fundamental immunology; genetically modified organisms; Human subjects; Humans; immunoglobulin E; Immunoglobulin E - metabolism; Incidence; Infant; Job syndrome; Job Syndrome - epidemiology; Job Syndrome - genetics; Job Syndrome - immunology; Male; mast cell; mast cells; Mast Cells - immunology; Medical sciences; Mice; Mice, Inbred C57BL; Mice, Transgenic; Middle Aged; morphine; Mutation; Mutation - genetics; Pathogenesis; patients; phenotype; phosphorylation; Plasma; risk; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; signal transducer and activator of transcription 3; signal transduction; Signal Transduction - genetics; STAT3 Transcription Factor - genetics; STAT3 Transcription Factor - physiology; Studies; transcription factors; Transgenes - genetics; volunteers; Young Adult; AD-HIES; Job syndrome; DNP; STAT3; mast cell; signal transducer and activator of transcription 3; NIH; BSB; Autosomal dominant hyper-IgE syndrome; PE; shRNA; degranulation; ERK; Extracellular signal-regulated kinase; National Institutes of Health; Basophile stimulation buffer; Phycoerythrin; 2,4-Dinitrophenol; Short hairpin RNA; Human; Allergy; Immunopathology; Hyperimmunoglobulinemia E syndrome; Immune deficiency; Genetic disease; Signal transduction; Immunology; Transcription factor STAT3; Degranulation; Genetics; Mast cell; Mutation; Signal transducer and activator of transcription 3

Severe atopic conditions associated with elevated serum IgE are heterogeneous with few known causes. Nearly every patient with autosomal-dominant hyper-IgE syndrome (AD-HIES) due to signal transducer and activator of transcription 3 (STAT3) mutations has a history of eczematous dermatitis and elevat...