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Alpha-1-proteinase inhibitor
- indication:For treatment of panacinar emphysema
- pharmacologypharmacology:
- mechanism: Alpha-1 proteinase inhibitor is a serine protease inhibitor (Serpin). Its primary mechanism is inhibiting the action of the serine protease called elastase (also plasmin and thrombin) in the lungs. The reactive center loop (RCL) of alpha-1 proteinase inhibitor extends out from the body of the protein and directs binding to the target protease. The protease cleaves the serpin at the reactive site, establishing a covalent linkage between the carboxyl group of the serpin reactive site and the serine hydroxyl of the protease. The resulting inactive serpin-protease complex is highly stable.
- toxicity:
- absorprion:
- halflife:
- roouteelimination:
- volumedistribution: * 5632 ± 2006 mL [ARALAST NP] * 5618 ± 1618 mL [Aralast]
- clearance: * 940 +/- 275 mL/day [Patients with congenital α1-PI deficiency with single IV infusion of 60 mg/kg]