Correlation between ABCA3 and Pediatric Pulmonary Disease
10.3969/j.issn.1000-3606.2018.03.015
- VernacularTitle:腺苷三磷酸结合盒转运体A家族成分3与儿科肺疾病相关性研究进展
- Author:
Xiaofei YUE
1
Author Information
1. 内蒙古医科大学附属医院新生儿科
- Keywords:
pulmonary surfactant;
ABCA3 gene;
neonatal respiratory distress syndrome;
pulmonary interstitial disease;
Lamellar body
- From:
Journal of Clinical Pediatrics
2018;36(3):227-230
- CountryChina
- Language:Chinese
-
Abstract:
Pulmonary surfactant is a lipid-protein complex that lines and stabilizes the respiratory interface in the alveoli, allowing for gas exchange during the breathing cycle. ATP-binding Cassette transporters A3, is a lipid transporter in the limiting membrane of lamellar bodies in alveolar type Ⅱ cells that plays a critical role in the regulation of pulmonary surfactant homeostasis. Mutations in the ABCA3 gene cause respiratory distress syndrome in new-born and childhood interstitial lung disease. An updated view on expression of ABCA3 gene and ABCA3 mutation associated with neonatal respiratory distress syndrome,or childhood interstitial lung disease was reviewed.
