Role of alpha 1-antitrypsin

Question 1

what inflammatory cells release elastase?

Answer 1

neutrophils and alveolar macrophages

Question 2

what is elastin normally inhibited by?

Answer 2

alpha 1 antitrypsin

Question 3

as inflammation persists, what happens to alpha 1 antitrypsin activity?

Answer 3

it decreases

Question 4

since alpha 1 antitrypsin is decreased in inflammation, what gets destructed in the lung?

Answer 4

elastic fibres, resulting in emphysema

Question 5

what happens when you have a inherited alpha 1 antitrypsin deficiency?

Answer 5

there is a limited amount of alpha 1 antitrypsin to counteract the elastatse released

Question 6

what is the amount of alpha 1 antitrypsin determined by?

Answer 6

by a pair of codominant genes named protein inhibitor genes

Question 7

how many mutations of a gene are there?

Answer 7

more than 75 mutations which are inherited as an autosomal recessive disorder

Question 8

what is the most serious alpha 1 antitrypsin deficiency caused by?

Answer 8

PIZ varient which is found in 5% of the population