Alpha 1 antitrypsinin the livers of patients with emphysema

Abstract

Parenchymal liver cells from emphysema patients with an inherited deficiency of alpha(1)-antitrypsin contain globules of glycoprotein that bind fluorescent antibody to alpha(1)-antitrypsin. The globules can be seen after hematoxylin and eosinstaining or on electron microscopy, but are more readily demonstrated by PAS stain of amylase-treated liver sections. It appears that an inappropriately large amount of alpha(1)-antitrypsin is found in the liver even when there is a deficiency in the serum. Genetic variants of the normal antitrypsin molecule may be unable to leave their site of synthesis in the liver cell because of some molecular aberration.