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Article

Alpha-1-antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model.

Hepatology (Baltimore, Md.) - 1 Oct 2007

Lindblad Douglas, Blomenkamp Keith, Teckman Jeffrey

Abstract excerpt

UNLABELLED: Alpha-1-antitrypsin (a1AT) deficiency is caused by homozygosity for the a1AT mutant Z gene and occurs in 1 in 2000 births. The Z mutation confers an abnormal conformation on the protein, resulting in an accumulation within the endoplasmic reticulum of hepatocytes rather than appropriate secretion. The accumulation of the mutant protein is strikingly heterogeneous within the liver. Homozygous ZZ...

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PMID: 17886264
DOI: 10.1002/hep.21822

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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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