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人Pi型S、MMalton的α1-抗胰蛋白酶以及去唾液酸M蛋白的分解代谢率。

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

作者信息

Jeppsson J O, Laurell C B, Nosslin B, Cox D W

出版信息

Clin Sci Mol Med. 1978 Jul;55(1):103-7. doi: 10.1042/cs0550103.

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.
摘要
  1. 从Pi型M、S和MMalton的受试者中分离出具有保留微异质性的人α1-抗胰蛋白酶。M蛋白被部分(20%)和完全去唾液酸化。这些蛋白质用125I或131I进行标记。2. 将两种标记蛋白同时注射到Pi M受试者体内后,研究了这些α1-抗胰蛋白酶的消失率。S蛋白和MMalton蛋白的分数分解代谢率分别为0.36和0.34天-1,而M蛋白为0.28天-1。S蛋白的血管外池与血浆池的比例为1.4,MMalton蛋白为1.6。20%去唾液酸化的M蛋白的分数分解代谢率增加了约100%。完全去唾液酸化的α1-抗胰蛋白酶的消失率极快。3. S蛋白的分数分解代谢率略高于M蛋白,这只能部分解释Pi型S受试者血浆浓度低40%的原因。同样,Pi MMalton蛋白分解代谢率的轻微增加太小,无法解释为什么Pi MMalton受试者血液中的α1-抗胰蛋白酶含量仅为正常水平的15%。肝脏分泌不足似乎是Pi S型和MMalton型受试者中α1-抗胰蛋白酶浓度低的主要原因,就像Pi Z型一样。

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