国际妇产科学杂志

国际妇产科学杂志 ›› 2009, Vol. 36 ›› Issue (5): 405-407.

• 综述 • 上一篇    下一篇

ATP连接盒转运子A3的研究进展

马小静;徐永萍   

  1. 250033 济南，山东大学第二医院妇产科
  • 收稿日期:2009-01-05 修回日期:1900-01-01 出版日期:2009-10-15 发布日期:2009-10-15

Research Advancement of ABCA3

MA Xiao-jing;XU Yong-ping   

  1. Department of Obstetrics and Gynecology, Second Hospital of Shandong University, Jinan 250033, China
  • Received:2009-01-05 Revised:1900-01-01 Published:2009-10-15 Online:2009-10-15

PDF (PC)

4773

被引次数

5

摘要： ATP连接盒（ABC）转运子A3（ABCA3）属于ABC转运子家族中ABCA亚家族的成员，其基因由超过80 000个核苷酸碱基组成，位于
16p13.3，可转录成大约6 500 bp的mRNA，直接翻译形成1 704个氨基酸组成的190 ku大小的蛋白质，即ABCA3蛋白。ABCA3蛋白在肺组织中高度表
达，参与脂类运输。ABCA3除与肿瘤细胞的多药耐药性有关外，其异常还可导致ABCA3蛋白的表达缺乏、减少或功能异常，影响肺表面活性蛋白B
（SP-B）和SP-C的加工、转运过程，破坏肺泡表面活性物质的动态平衡，导致新生儿急性呼吸窘迫综合征，增加肺间质性疾病的发生。就ABCA3
的研究现状做简要综述。

关键词: ATP连接盒转运子A3, 新生儿, 板层小体, 肺疾病

Abstract: The ABCA3 gene, of the ABCA subclass of ATP binding cassette （ABC） transporters, is expressed
exclusively in lung and is located on chromosome 16p13.3. It spans over 80 000 nucleotide bases and is transcribed into an
approximately 6 500-bp mRNA, which directs the synthesis of a 1704-amino-acid protein of 190 kDa size. ABCA3 mediates the
translocation of a wide variety of substrates, including lipids, across cellular membranes. It does not only cause multidrug
resistance but also causes a neonatal respiratory distress syndrome and increase susceptibility to milder forms of
interstitial lung disease. ABCA3 variants might result deficiency or reduction of ABCA3 protein expression. Sometimes the
protein expressed in the correct amount and location, but does not function properly. These conditions may influence the
process and transportion of surfactant proteins B and C, break the pulmonary surfactant phospholipid homeostasis, and induce
to the neonatal lung diseases.

Key words: ABCA3, Newborn, Lamellar body, Lung disease