Alpha-1 Antitrypsin:
Chapter: 12
(also known as protease inhibitor, or Pi) is a circulating glycoprotein synthesized in the liver, which normally acts to inhibit proteolytic enzymes such as trypsin, elastase, and collagenase which are capable of destroying lung tissue. Patients with alpha-1 antitrypsin deficiency have low antiproteolytic activity and are at high risk for the development of COPD, usually panlobular emphysema. The defect is genetic, and alpha-1 antitrypsin is the product of two allelles located on chromosome 14. A normal individual is pimm; homozygotes are pizz; heterozygotes are pimz.