STAT3 Mutation-Associated Airway Epithelial Defects in Job Syndrome.

Yihan Zhang,Tian Lin,Hui Min Leung,Cheng Zhang, Brittany Wilson-Mifsud,Michael B. Feldman,Anne Puel,Fanny Lanternier,Louis-Jean Couderc,Francois Danion,Emilie Catherinot,Hélène Salvator,Colas Tcherkian, Claire Givel,Jie Xu,Guillermo J. Tearney,Jatin M. Vyas,Hu Li,Bryan P. Hurley,Hongmei Mou

The Journal of allergy and clinical immunology（2023）

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摘要

AD-HIES STAT3 mutations confer numerous abnormalities to airway epithelial cells in cell differentiation and host innate immunity, emphasizing their involvement in the pathogenesis of lung complications in Job Syndrome. Therefore, therapies must address the epithelial defects as well as the previously noted immune cell defects to alleviate chronic infections in Job Syndrome patients.

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关键词

AD-HIES STAT3 Mutation,Cell Differentiation,Innate Immunity,Mucociliary Clearance,Neutrophil Chemotaxis

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