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[α1抗胰蛋白酶缺乏症与肝脏疾病]

[Alpha 1 antitrypsin deficiency and liver diseases].

作者信息

Brochard C

出版信息

Sem Hop. 1975 Mar 26;51(15):1019-24.

PMID:175465
Abstract

Alpha-1 antitrypsin is mainly responsible for the inhibitory power with regard to serum of numerous enzymes, in particular, trypsin. Alpha-1 antitrypsin deficiency is linked to pulmonary and hepatic disease. The special characteristic constantly found during liver disease, associated with alpha-1 antitrypsin deficiency, is the existence of intra-cytoplasmic globules within the hepatocytes due to accumulation of alpha-1 antitrypsin. This latter enzyme differs chemically from serum alpha-1 antitrypsin.

摘要

α-1抗胰蛋白酶主要负责对血清中多种酶,特别是胰蛋白酶的抑制作用。α-1抗胰蛋白酶缺乏与肺部和肝脏疾病有关。在与α-1抗胰蛋白酶缺乏相关的肝脏疾病中经常发现的特殊特征是,由于α-1抗胰蛋白酶的积累,肝细胞内存在细胞质内小球。后一种酶在化学上与血清α-1抗胰蛋白酶不同。

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