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Thorax. Jun 2004; 59(6): 529–535.
PMCID: PMC1747018
α1-Antitrypsin deficiency • 4: Molecular pathophysiology
D Lomas and H Parfrey
The molecular basis of α1-antitrypsin deficiency is reviewed and is shown to be due to the accumulation of mutant protein as ordered polymers within the endoplasmic reticulum of hepatocytes. The current goals are to determine the cellular response to polymeric α1-antitrypsin and to develop therapeutic strategies to block polymerisation in vivo.
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