STAT3 mutation-associated airway epithelial defects in Job syndrome.

ABSTRACT:

Background

Job syndrome is a disease of autosomal dominant hyper-IgE syndrome (AD-HIES). Patients harboring STAT3 mutation are particularly prone to airway remodeling and airway infections.

Objectives

Airway epithelial cells play a central role as the first line of defense against pathogenic infection and express high levels of STAT3. This study thus interrogates how AD-HIES STAT3 mutations impact the physiological functions of airway epithelial cells.

Methods

This study created human airway basal cells expressing 4 common AD-HIES STAT3 mutants (R382W, V463del, V637M, and Y657S). In addition, primary airway epithelial cells were isolated from a patient with Job syndrome who was harboring a STAT3-S560del mutation and from mice harboring a STAT3-V463del mutation. Cell p

SUBMITTER: Zhang Y 

PROVIDER: S-EPMC10330947 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature