Pulmonary surfactant homeostasis associated genetic abnormalities and lung diseases / 中华医学遗传学杂志
Chinese Journal of Medical Genetics
;
(6): 564-568, 2016.
Article
in Chinese
| WPRIM
| ID: wpr-345408
ABSTRACT
Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis. This paper has provided a review for the functions and processes of pulmonary surfactant metabolism, as well as the connection between disorders of surfactant homeostasis genes and lung diseases.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Transcription Factors
/
Pulmonary Surfactants
/
ATP-Binding Cassette Transporters
/
Pulmonary Surfactant-Associated Protein C
/
DNA-Binding Proteins
/
Genetics
/
Homeostasis
/
Lung Diseases
/
Metabolism
Limits:
Humans
Language:
Chinese
Journal:
Chinese Journal of Medical Genetics
Year:
2016
Type:
Article
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