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Plasma glycoprotein member of the serpin superfamily which inhibits
trypsin, neutrophil elastase, and other proteolytic enzymes. Commonly referred to
as alpha 1-proteinase inhibitor (A1PI), it exists in over 30 different biochemical
variant forms known collectively as the Pi (protease inhibitor) system.
Hereditary A1PI deficiency is associated with pulmonary emphysema.
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References:
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Pharmacologic Action
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Serine Proteinase Inhibitor
(Exogenous or endogenous compounds which inhibit serine endopeptidases.)
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Trypsin Inhibitor
(Serine proteinase inhibitors which inhibit trypsin. They may be endogenous or exogenous compounds.)
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Alpha 1-Antitrypsin Deficiency
Deficiency of the protease inhibitor Alpha 1-Antitrypsin, leading primarily to degradation of elastin of the alveolar walls, as well as other structural proteins of a variety of tissues.
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Pulmonary Emphysema
A subcategory of chronic obstructive pulmonary disease. The disease is characterized by anatomic alterations of the lungs, such as the enlargement of airspaces and destruction of alveolar walls.
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