Catabolic rate of alpha1-antitrypsin of of Pi types S, and MMalton and of asialylated M-protein in man.

J O Jeppsson, C B Laurell, B Nosslin, D W Cox
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引用次数: 26

Abstract

1. Human alpha1-antitrypsin was isolated with preserved microheterogeneity from subjects of Pi types M, S and MMalton. The M-protein was partially (20%) and completely desialylated. The proteins were labelled with either 125I or 131I. 2. The disappearance rate of these alpha1-antitrypsins was studied after simultaneous injection of the two types of labelled protein into Pi M subjects. The fractional catabolic rates of S- and MMalton-protein were 0.36 and 0.34 day(-1) respectively compared with 0.28 day(-1) for M-protein. The ratio of extravascular to plasma pools was 1.4 for S- protein and 1.6 for MMalton-protein. The 20% desialylated M-protein showed an increase of about 100% in its fractional catabolic rate. The disappearance rate of completely desialylated alpha1-antitrypsin was extremely rapid. 3. The slightly higher fractional catabolic rate of S- than of M-protein can only partly explain the 40% lower plasma concentration in subjects of Pi type S. Similarly the slight increase in catabolic rate of Pi MMalton- protein is too small to explain why the alpha1-antitrypsin content of the blood in Pi MMalton subjects is only 15% of that normally found. A low hepatic secretion seems to be the major cause of the low alpha1-antitrypsin concentration found in subjects of types Pi S and MMalton, as in Pi type Z.

α - 1-抗胰蛋白酶在人体内的分解代谢率。
1. 从Pi型M、S和MMalton患者中分离到具有显微异质性的人α - 1抗胰蛋白酶。m蛋白部分(20%)和完全脱氮。蛋白质用125I或131I标记。2. 将这两种标记蛋白同时注射到Pi M受试者体内后,研究了这两种α - 1抗胰蛋白酶的消失率。S-和mmalton蛋白的分解代谢率分别为0.36和0.34 d (-1), m蛋白的分解代谢率为0.28 d(-1)。S-蛋白和mmalton蛋白的血管外池与血浆池之比分别为1.4和1.6。20%脱氮m蛋白的部分分解代谢率增加了约100%。完全去乙酰化的α - 1抗胰蛋白酶消失速度极快。3.S-蛋白的分解率略高于m -蛋白,这只能部分解释π S型患者血浆浓度降低40%的原因。同样,π MMalton-蛋白的分解率的轻微增加也不足以解释π MMalton患者血液中α - 1抗胰蛋白酶含量仅为正常水平的15%的原因。低肝分泌似乎是在Pi S型和MMalton型受试者中发现的低α - 1抗胰蛋白酶浓度的主要原因,就像在Pi Z型中一样。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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