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alpha1 protease inhibitor deficiency

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Alpha-1 antitrypsin is a member of the serine protease inhibitor family of plasma proteins.

Alpha-1 antitrypsin has a wide spectrum of antiprotease activity, however, its important physiological role is the inhibition of neutrophil elastase.

Alpha-1 antitrypsin is primarily synthesised in the liver and normally constitutes about 90% of the circulating blood alpha-1 globulin.

Severe alpha-1 antitrypsin deficiency is associated with lung and liver disease.


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