Article
STAT3-Dependent Regulation of CFTR and Ciliogenesis Is Essential for Mucociliary Clearance and Innate Airway Defense in Hyper-IgE Syndrome.
American journal of respiratory and critical care medicine - 1 Oct 2025
Sun Ling, Walls Samantha A, Dang Hong, Quinney Nancy L, Sears Patrick R, Sadritabrizi Taraneh, Hasegawa Koichi, Okuda Kenichi, Asakura Takanori, Chang Xiuya, Zheng Meiqi, Mikami Yu, Dizmond Felicia U, Danilova Daniela, Zhou Lynn, Deshmukh Anshulika, Cholon Deborah M, Radicioni Giorgia, Rogers Troy D, Kissner William J, Markovetz Matthew R, Guhr Lee Tara N, Gutay Mark I, Esther Charles R, Chua Michael, Grubb Barbara R, Ehre Camille, Kesimer Mehmet, Hill David B, Ostrowski Lawrence E, Button Brian, Gentzsch Martina, Robinson Chevalia, Olivier Kenneth N, Freeman Alexandra F, Randell Scott H, Vladar Eszter, O'Neal Wanda K, Boucher Richard C, Chen Gang
Abstract excerpt
Rationale: Hyper-IgE syndrome (STAT3-HIES), also known as Job's syndrome, is a rare immunodeficiency disease typically caused by dominant-negative STAT3 mutations. STAT3-HIES is characterized by chronic pulmonary infection and inflammation, suggesting impaired innate host defense. Objectives: To identify airway epithelial host defense defects caused by STAT3 mutations that, together with immune dysfunction,...
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