腺苷三磷酸结合盒转运体A家族成分3（ABCA3）表达于肺泡Ⅱ型上皮细胞中层状体的外膜，在维持肺表面活 性物质的稳态中起至关重要的作用，ABCA3基因的异常表达与新生儿呼吸窘迫综合症、儿童间质性肺疾病的发生有着密切的 联系。现就ABCA3基因的表达、ABCA3的生理功能、ABCA3基因的突变与儿科肺部疾病的关系的研究现状作一综述。

　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.