What is the most abundant alpha-1 globulin? What is its function? What is the clinical result of its non-functioning?
1. Alpha-1 antiprotease (aka antitrypsin)
2. Alpha-1 antiprotease AKA antitripsin INHIBITS elastase, an enzyme that breaks down the CT protein known as elastin (a component of lung wall)
3. Pts who are homozygous for a genetic defect in elastin are at high risk for developing emphysema
4. cigarette smoke modifies an alpha 1 antiprotease methionine to form a sulfoxide, rendering it much less active. thereby inhibiting less elastase, and leading to move broken down CT and emphysema!