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Updated: Nov 9, 2025

Generation of Airway Epithelial Cell Air-Liquid Interface Cultures from Human Pluripotent Stem Cells
Published on: June 14, 2022
Generation of an induced pluripotent stem cell line from a patient with surfactant metabolism dysfunction carrying
Xiaojuan Yin1, Danhua Zhao2, Zhichen Tian2
1Bayi Children's Hospital, The Seven Medical Center of PLA General Hospital, Beijing 100700, China; Department of Pediatrics, Hainan Hospital of the PLA General Hospital, Sanya, Hainan Province 572000, China; The Second School of Clinical Medicine, Southern Medical University, Guangzhou Province 510280, China.
Abstract:
Surfactant dysfunction is a genetically heterogeneous pulmonary disease that causes dyspnea. ATP binding cassette protein transporter subunit A3 (ABCA3) is the main pathogenic gene of pulmonary surfactant dysfunction. In this study, we established an induced pluripotent stem cell line (SMCPGHi001-A) from the peripheral blood cells of a 49-day-old male infant, carrying compound heterozygous variations of the ABCA3 gene (c.3997_3998del, p.R1333fs, and c.3137C > T, p.A1046V). This iPSC line would be a useful tool to study the pathogenesis, disease development, and treatment of pulmonary surfactant dysfunction.
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