Alpha-I-antitrypsin and copper in the liver.
The incidental finding of orcein positive granules, indicating copper associated protein, in alpha-1-antitrypsin (AAT) positive liver biopsies stimulated a histochemical search for evidence of copper and copper-binding protein in a series of 46 liver biopsies with histological evidence of AAT accumulation. Hepatic accumulation copper and copper-binding protein occurred in all 19 cirrhotics (100%) and in 14 out of 27 non-cirrhotic livers (51.85%). The overall percentage was 71.73%. AAT and copper deposits coexisted in the same periportal hepatocytes. AAT globules showed positive reactivity both to rhodanine and orcein stains. The severity of chronic liver damage correlated with increasing amounts of copper deposition. It is suggested that in AAT storage, not only is the metabolism of this substance disturbed, but also that of proteins involved in copper metabolism and excretion, resulting in copper accumulation within hepatocytes.
D000293:Adolescent; D000328:Adult; D000368:Aged; D002352:Carrier Proteins / Q000032:analysis; D002780:Cholestasis, Intrahepatic / Q000378:metabolism; D003300:Copper / Q000032:analysis*; D003594:Cytoplasmic Granules / Q000032:analysis; D005260:Female; D006801:Humans; D008099:Liver / Q000032:analysis*; D008103:Liver Cirrhosis / Q000378:metabolism*; D008107:Liver Diseases / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D012236:Rhodanine; D000515:alpha 1-Antitrypsin / Q000032:analysis*
D002352:Carrier Proteins; D000515:alpha 1-Antitrypsin; C023548:copper-binding protein; D003300:Copper; D012236:Rhodanine