Immunologic studies of arginase in tissues of normal human adult and arginase-deficient patients.
Rabbit antibody to human liver arginase was used to examine the immunologic characteristics of arginase in red blood cells (RBC), liver, kidney, brain, and gastrointestinal tract from normal adults and from patients with hyperargininemia. Greater than 90% of the arginase in RBC and liver was precipitated by this antibody whereas only 50% of the arginase in kidney, brain, and gastrointestinal tract reacted with it. Two siblings and a third patient with hyperargininemia were found to have immunoreactive arginase protein in their RBC that was enzymatically inactive. The amount of arginase protein approximated that found in RBC from normal individuals. A kidney biopsy obtained from one of the patients with hyperargininemia had arginase activity 4-5-fold greater than that found in normal kidney biopsy material. Double immunodiffusion and precipitation-inhibition studies demonstrated two types of arginase protein in this patient's kidney: one enzymatically inactive and precipitated by the antibody, and one enzymatically active but not precipitated by the antibody. These data, in conjunction with biochemical data reported previously demonstrate that there are two gene loci determining arginase in man.
D000592:Amino Acid Metabolism, Inborn Errors / Q000201:enzymology* / Q000235:genetics; D001119:Arginase / Q000235:genetics / Q000276:immunology*; D001120:Arginine / Q000097:blood*; D001921:Brain / Q000201:enzymology; D002874:Chromosome Mapping; D004064:Digestive System / Q000201:enzymology; D004912:Erythrocytes / Q000201:enzymology; D005796:Genes; D006801:Humans; D020162:Hyperargininemia; D005779:Immunodiffusion; D007668:Kidney / Q000201:enzymology
D001120:Arginine; D001119:Arginase