title: Succinylacetone and delta-aminolevulinic acid dehydratase in hereditary tyrosinemia: immunochemical study of the enzyme.
abstract: Immunochemical determinations of delta-aminolevulinic acid (ALA) dehydratase were performed in erythrocytes and in liver of a patient with hereditary tyrosinemia who underwent liver transplantation for correction of this metabolic disorder. Both erythrocytic and hepatic ALA dehydratase activities were extremely low before liver transplantation, but they appeared normal after transplantation. According to results of immunochemical quantification of ALA dehydratase, the level of the enzyme protein in erythrocytes was not different before, during, and after liver transplantation. Immunoquantifiable enzyme concentrations were not substantially different in the patient's own liver as compared with the transplanted liver. These findings indicate that although succinylacetone, an abnormal metabolite produced in tyrosinemia, is a potent inhibitor of the activity of ALA dehydratase, it has a far less effect on the synthesis of the enzyme protein.
authors.affiliation: Rockefeller University Hospital, New York, New York.
authors.forename: S
authors.initials: S
authors.lastname: Sassa
authors.forename: H
authors.initials: H
authors.lastname: Fujita
authors.forename: A
authors.initials: A
authors.lastname: Kappas
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000201:enzymology* / Q000601:surgery; D002675:Child, Preschool; D004591:Electrophoresis, Polyacrylamide Gel; D004912:Erythrocytes / Q000032:analysis / Q000201:enzymology; D006537:Heptanoates / Q000378:metabolism*; D006538:Heptanoic Acids / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000032:analysis / Q000201:enzymology; D016031:Liver Transplantation; D008297:Male; D000623:Porphobilinogen Synthase / Q000032:analysis / Q000378:metabolism*; D011863:Radioimmunoassay; D014443:Tyrosine / Q000097:blood*
chemical_list: D006537:Heptanoates; D006538:Heptanoic Acids; D014443:Tyrosine; C020804:succinylacetone; D000623:Porphobilinogen Synthase