α1-antitrypsin deficiency•4:: Molecular pathophysiology

被引：112
作者：
Lomas, DA [1 ]
Parfrey, H [1 ]
机构：
[1] Univ Cambridge, Cambridge Inst Med Res, Dept Med, Resp Med Unit, Cambridge CB2 2XY, England
来源：
THORAX | 2004年 / 59卷 / 06期
关键词：
D O I：
10.1136/thx.2003.006528
中图分类号：
R56 [呼吸系及胸部疾病];
学科分类号：
摘要：
The molecular basis of alpha(1)-antitrypsin deficiency is reviewed and is shown to be due to the accumulation of mutant protein as ordered polymers within the endoplasmic reticulum of hepatocytes. The current goals are to determine the cellular response to polymeric alpha(1)-antitrypsin and to develop therapeutic strategies to block polymerisation in vivo.
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收藏
页码：529 / 535
页数：7
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