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α 1 -Antitrypsin (α1AT) provides the major protection in the lung against neutrophil elastase-mediated proteolysis. Inheritance of α1AT deficiency alleles is associated with an increased risk of emphysema and liver disease. α1AT null alleles cause the total absence of serum α1AT and represent the ultimate in a continuum of alleles associated with α1AT deficiency. The molecular mechanisms responsible...