中国输血杂志2017，Vol.30Issue(3)：316-319,4.DOI:10.13303/j.cjbt.issn.1004-549x.2017.03.027
α1-抗胰蛋白酶相关疾病及其临床应用进展
Research progress on related diseases and clinical application of α1-antitrypsin
摘要
Abstract
α1-antitrypsin (AAT),an human plasma protease inhibitor,plays an important role in protecting the normal cells and organs from protease damage.α1-antitrypsin deficiency (AATD) is a common single-gene disease.The patients with AATD may develop emphysema,cirrhosis and other diseases,even are at risk of death.AAT replacement therapy with intravenous infusions of purified AAT from human plasma is the most usual clinical treatment method for AATD to elevate the serum AAT level and partially recover the biochemical defect.In recent years,several new therapeutic methods for AATD,including the inhaled AAT,gene therapy and stem cell therapy,have been developed.In the present paper,the molecular characteristics,the functional mechanism of AAT and its related diseases were described.In addition,the application of AAT in the AATD-related diseases was reviewed,and the technological development and market prospects of AAT product were studied.关键词
α1-抗胰蛋白酶/α1-抗胰蛋白酶缺乏症(AATD)/临床应用/研究进展Key words
α1-antitrypsin/alpha-1-antitrypsin deficiency (AATD)/clinical application/research progress分类
医药卫生引用本文复制引用
张剑平,周兰贞,郭采平..α1-抗胰蛋白酶相关疾病及其临床应用进展[J].中国输血杂志,2017,30(3):316-319,4.基金项目
深圳重组血浆蛋白类创新药物工程实验室项目 （）
