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Updated: Aug 19, 2025
09:45
Generation of 3D Whole Lung Organoids from Induced Pluripotent Stem Cells for Modeling Lung Developmental Biology and Disease
Published on: April 12, 2021
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Congenital lung malformations: Dysregulated lung developmental processes and altered signaling pathways
Fabian Doktor1, Lina Antounians1, Martin Lacher2
1Developmental and Stem Cell Biology Program, Peter Gilgan Centre for Research and Learning, The Hospital for Sick Children, Toronto, Canada; Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Canada.
Seminars in Pediatric Surgery
|November 28, 2022
Summary
Congenital lung malformations, including CPAM and BPS, have poorly understood molecular pathways. Recent advances in animal models and sequencing offer new insights into their development and potential malignant transformation.
Area of Science:
- Pulmonology
- Developmental Biology
- Molecular Pathology
Background:
- Congenital lung malformations (CLMs) encompass diverse conditions like CPAM, BPS, CLE, and bronchogenic cysts.
- The underlying signaling pathways and malignant transformation potential of CLMs remain largely unknown.
- Recent advancements in animal models and next-generation sequencing have begun to elucidate CLM pathophysiology.
Purpose of the Study:
- To provide an overview of normal human and rodent lung development.
- To discuss the current understanding of the pathophysiology of various CLMs.
- To explore the molecular pathways altered in congenital lung malformations.
Main Methods:
- Review of normal lung development in humans and rodents.
- Analysis of current literature on CLM pathophysiology.
- Integration of findings from transgenic/knockout animal models and next-generation sequencing studies.
Main Results:
- Detailed overview of normal lung development processes.
- Discussion of altered molecular pathways in CPAM, BPS, CLE, and other CLMs.
- Identification of knowledge gaps in CLM pathogenesis and malignant transformation.
Conclusions:
- Understanding normal lung development is crucial for deciphering CLM origins.
- Molecular insights from recent studies are enhancing our knowledge of CLM pathophysiology.
- Further research into signaling pathways is needed to address malignant transformation risks.
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