Emphysema inherited

The protective antiprotease -antitrypsin (AAT) inhibits several protease enzymes, including neutrophil elastase. In the presence of unopposed AAT activity, elastase attacks elastin, which is a major component of alveolar walls. A hereditary deficiency of AAT results in an increased risk for premature development of emphysema. In the inherited disease, there is an absolute deficiency of AAT. In emphysema resulting from cigarettesmoking, the imbalance is associated with increased protease activity or reduced activity of antiproteases. Activated inflammatory cells release several other proteases, including cathepsins and metalloproteinases. In addition, oxidative stress reduces antiprotease (or protective) activity. 

Human neutrophil elastase is the proteinase that has received by for the most attention over the last 30 years primarily due to the foci that it was thought to be the rntyor factor involved in the development of emphysema. This assumption was derived from the observation of Eriksson In 1963 that individuals with an inherited deficiency of cti-PI developed emphysema at an early age [11]. One year later. Gross showed that emphysema could be produced in experimental animals after intratracheal administration of papain, a plant proteinase with a... 

Precautions Moderately toxic by breathing. A human mutagen (changes inherited characteristics). Some respiratory system effects by breathing are changes in the trachea or bronchi, emphysema, chronic pulmonary edema or congestion. A strong irritant to eyes, nose, and throat. 

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