Purification and Partial Characterization of PAS-Positive Inclusion Bodies from the Liver in Alpha1-Antitrypsin Deficiency

Abstract

To characterize the periodic acid-Schiffpositive inclusion bodies from cirrhotic livers of patients with homozygous alpha1-antitrypsin deficiency we isolated and analyzed such material. The main component of the inclusion bodies was a protein with approximately the same molecular size as serum alpha1-antitrypsin. Immunologic similarity between the isolated protein and serum alpha1-antitrypsin was established by the isolate's ability to react with fluorescein-isothiocyanate-conjugated antibodies against serum alpha1-antitrypsin and to raise antibodies gainst it in rabbits, and with the double immunodiffusion technic.