Related Experiment Video
Updated: Jul 13, 2026
08:46
A Standardized Method for Measuring Internal Lung Surface Area via Mouse Pneumonectomy and Prosthesis Implantation
Published on: July 26, 2017
Lung development and adult lung diseases.
Wei Shi1, Saverio Bellusci, David Warburton
1Developmental Biology Program, Saban Research Institute, Children's Hospital Los Angeles, 4650 Sunset Blvd MS35, Los Angeles, CA 90027, USA.
Chest
|August 19, 2007
Summary
Genetic factors significantly influence adult respiratory diseases by affecting lung development and repair. Understanding these genetic links is crucial for identifying susceptibility to conditions like COPD, cystic fibrosis, and asthma.
Area of Science:
- Pulmonary Medicine
- Genetics
- Developmental Biology
Background:
- Adult respiratory diseases stem from complex genetic-environmental interactions impacting lung development and repair.
- Abnormal lung structure and function, influenced by genetics, can predispose individuals to diseases such as COPD, cystic fibrosis (CF), and asthma.
- While smoking is a major risk factor for COPD, genetic susceptibility plays a key role in disease onset and progression.
Purpose of the Study:
- To review the relationship between lung development and the pathogenesis of major adult respiratory diseases.
- To explore the genetic factors contributing to susceptibility and disease mechanisms in COPD, CF, and asthma.
- To discuss candidate genes and pathways involved in lung development and their implications for respiratory disease.
Main Methods:
- Literature review focusing on genetic-environmental interactions in lung development.
- Analysis of studies investigating gene function and its impact on lung structure and function in animal models.
- Examination of genetic factors implicated in the pathogenesis of COPD, CF, and asthma.
Main Results:
- Genetic abnormalities affect fetal lung development, postnatal maturation, and adult lung injury/repair.
- Mouse models demonstrate that genetic alterations can lead to respiratory distress, reduced alveolarization, or early-onset emphysema.
- Candidate genes (e.g., elastin, lysyl oxidase, fibrillin, TGF-β/Smad3 pathway) and extracellular matrix proteases are discussed as susceptibility factors.
- CFTR gene mutations impact fetal lung epithelial cell differentiation, leading to subtle structural/functional abnormalities in infants.
- Airway remodeling due to inflammation in infancy is linked to later asthma development.
Conclusions:
- Lung development is intrinsically linked to the pathogenesis of adult respiratory diseases.
- Genetic susceptibility is a critical determinant for developing conditions like COPD, CF, and asthma.
- Further research into developmental genetics is essential for understanding and potentially preventing adult lung diseases.
Related Concept Videos
01:24Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features
Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
01:17Other Pulmonary Disorders
Respiratory disorders encompass a range of conditions with varying levels of severity. Asthma, marked by chronic airway inflammation and hypersensitivity, is one such condition. It can lead to airway obstruction due to factors like bronchial spasms, mucosal edema, increased mucus secretion, or epithelial damage. Asthma triggers are diverse, ranging from allergens to emotional upset, and treatment focuses on both immediate relief through bronchodilators and long-term inflammation suppression.
01:20Chronic Obstructive Pulmonary Disease-II: Pathophysiology
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
Chronic Inflammation
01:23Chronic Obstructive Pulmonary Disease II: Emphysema
Emphysema, a major phenotype of chronic obstructive pulmonary disease (COPD), is characterized by irreversible destruction of alveolar walls and permanent enlargement of distal airspaces. Unlike chronic bronchitis, which primarily affects the airways, emphysema predominantly involves the lung parenchyma, where structural damage leads to airflow limitation.PathophysiologyIt most commonly results from prolonged exposure to cigarette smoke and other toxic gases, particularly cigarette smoke.
01:23Chronic Obstructive Pulmonary Disease I: Introduction
Chronic obstructive pulmonary disease is a common, preventable, and treatable respiratory disorder characterized by persistent symptoms and progressive airflow limitation. This limitation results from a combination of small-airway disease (obstructive bronchiolitis) and parenchymal destruction (emphysema), both driven by chronic inflammation from exposure to harmful particles or gases.The disease includes two main pathological entities: emphysema, marked by destruction of alveolar walls and...
01:20COPD: Pathogenesis and Clinical Features
Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...