Loop-sheet polymerization: the structural basis of Z alpha 1-antitrypsin accumulation in the liver.

doi:10.1042/cs0860489

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Article

Loop-sheet polymerization: the structural basis of Z alpha 1-antitrypsin accumulation in the liver.

Clinical science (London, England : 1979) - 1 May 1994

Lomas D A

Abstract excerpt

1. The Z deficiency variant of alpha 1-antitrypsin predisposes the homozygote to early-onset panlobular emphysema and results in the accumulation of antitrypsin within the hepatocyte, which leads to hepatocellular damage and cirrhosis. The mechanism of this accumulation has been shown to be due t...

Topics

Humans
Liver
Macromolecular Substances
Mutation
Protein Conformation
Structure-Activity Relationship
alpha 1-Antitrypsin

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