alpha-1 antitrypsin is a serine protease inhibitor produced in the liver.
In the lung it prevents neutrophil elastase from breaking down the alveolar wall.
It is inactivated by cigarette smoke.
Genetic varients of alpha-1 antitrypsin exist and are labelled based on their electrophoresis mobility, with M (medium), S (slow) and Z (very slow). MM is 'normal' while 'ZZ' has the worst prognosis with panlobular emphysema.
Patients with an alpha-1 antitrypsin defiency are likely to develop COPD early (even without smoking) and liver cirrhosis is common.
Now for a very straightforward battle - cystic fibrosis
