Article
The molecular basis of alpha 1-antichymotrypsin deficiency in a heterozygote with liver and lung disease.
Journal of hepatology - 1 Jul 1993
Faber J P, Poller W, Olek K, Baumann U, Carlson J, Lindmark B, Eriksson S
Abstract excerpt
Alpha 1-antichymotrypsin (alpha 1-ACT) is a serine proteinase inhibitor (serpin) with cathepsin G, mast cell chymase and chymotrypsin as target enzymes. We present the case of a middle-aged man with low plasma levels of alpha 1-ACT, asthma with progression to emphysema, and chronic HCV positive liver disease with selective accumulation of alpha 1-ACT in hepatocytes. This secretory defect is analogous to that seen...
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