The molecular basis of α1-antichymotrypsin deficiency in a heterozygote with liver and lung disease
JP Faber, W Poller, K Olek, U Baumann, J Carlson… - Journal of …, 1993 - Elsevier
Alpha 1-antichymotrypsin (α 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 α 1-ACT, asthma with progression to emphysema, and
chronic HCV positive liver disease with selective accumulation of α 1-ACT in hepatocytes.
This secretory defect is analagous to that seen in Pi Z α 1-antitrypsin deficiency. The
molecular basis of α 1-ACT deficiency in this patient has been characterized by direct …
G, mast cell chymase and chymotrypsin as target enzymes. We present the case of a middle-
aged man with low plasma levels of α 1-ACT, asthma with progression to emphysema, and
chronic HCV positive liver disease with selective accumulation of α 1-ACT in hepatocytes.
This secretory defect is analagous to that seen in Pi Z α 1-antitrypsin deficiency. The
molecular basis of α 1-ACT deficiency in this patient has been characterized by direct …