Alpha-1-antitrypsin and copper in the liver

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Abstract
The incidental finding of orcein positive granules, indicating Cu associated protein, in .alpha.-I-antitrypsin (AAT) positive [human] liver biopsies stimulated a histochemical search for evidence of Cu and Cu-binding protein in a series of 46 liver biopsies with histological evidence of AAT accumulation. Hepatic accumulation of Cu and Cu-binding protein occurred in all 19 cirrhotics (100%) and in 14 out of 27 noncirrhotic livers (51.85%). The overall percentage was 71.73%. AAT and Cu deposits coexisted in the same periportal hepatocytes. ATT globules showed positive reactivity to rhodanine and orcein stains. The severity of chronic liver damage correlated with increasing amounts of Cu deposition. In ATT storage, the metabolism of this substance is apparently not only disturbed, but also that of proteins involved in Cu metabolism and excretion, resulting in Cu accumulation within hepatocytes.