期刊
CLINICS IN LIVER DISEASE
卷 22, 期 4, 页码 643-+出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.cld.2018.06.010
关键词
Alpha 1 antitrypsin; Autophagy; Proteolysis; ERAD; Protein polymer; siRNA
In homozygous ZZ alpha-1-antitrypsin (AAT) deficiency, the liver synthesizes large quantities of AAT mutant Z, which folds improperly during biogenesis and is retained within the hepatocytes and directed into intracellular proteolysis pathways. These intracellular polymers trigger an injury cascade, which can lead to liver injury. This process is highly variable, and not all patients develop liver disease. Although the injury cascade is not fully described, there is likely a strong influence of genetic and environmental modifiers of the injury cascade and of the fibrotic response. With improved understanding of liver injury mechanisms, new strategies for treatment are now being explored.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据