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  

Correlation between ABCA 3 and Pediatric Pulmonary Disease

作　　者：月小飞 梅花[1] 刘春枝[1] YUE Xiaofei;MEI Hua;LIU Chunzhi(Department of Neonatology, Affiliated Hospital, Inner Mongolia Medical University, Hohhot 010050, Inner Mongolia,China)

机构地区：[1]内蒙古医科大学附属医院新生儿科,内蒙古呼和浩特010050

出　　处：《临床儿科杂志》2018年第3期227-230,共4页Journal of Clinical Pediatrics

基　　金：国家自然科学基金项目(No.81260107);内蒙古自治区自然科学基金项目[No.2015MS(LH)0810;2011MS1111]

摘　　要：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 typeⅡcells that plays a critical role in the regulation of pulmonary surfactant homeostasis.Mutations in the ABCA3gene cause respiratory distress syndrome in new-born and childhood interstitial lung disease.An updated view on expression of ABCA3gene and ABCA3mutation associated with neonatal respiratory distress syndrome,or childhood interstitial lung disease was reviewed.

关 键 词：肺表面活性物质 ABCA3基因 新生儿呼吸窘迫综合征 肺间质疾病 板层小体 

分 类 号：R725.6[医药卫生—儿科]