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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 to the Z mutation (Glu-342-->Lys) perturbing the structure of the protein, allowing a unique interaction between the...

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Identifiers

PMID: 8033502
DOI: 10.1042/cs0860489

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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