1. Absence of erythrocyte arginase protein in Japanese patients with hyperargininemia
- Author
-
Takashi Murachi, H. Keino, Shigeo Kashiwamata, S. Aono, Naoki Mizutani, Masayuki Totani, C. Hayakawa, Kimi Watanabe, and Masaki Ikemoto
- Subjects
Adult ,Male ,Erythrocytes ,Arginine ,Immunoblotting ,Hyperargininemia ,Biology ,Japan ,medicine ,Humans ,Child ,Amino Acid Metabolism, Inborn Errors ,Gel electrophoresis ,chemistry.chemical_classification ,Arginase ,Human liver ,Hyperammonemia ,medicine.disease ,Molecular biology ,Red blood cell ,Enzyme ,medicine.anatomical_structure ,Biochemistry ,chemistry ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Electrophoresis, Polyacrylamide Gel ,Female - Abstract
In Japan, hyperargininemia has been reported in only 5 unrelated families and four patients are alive at present. In this study we examined arginase protein in erythrocytes of these Japanese patients using two analytical methods of immunoblotting and two-dimensional gel electrophoresis. Immunoblotting study with anti-E. coli-expressed human liver arginase rabbit IgG revealed lack of cross-reacting materials in the erythrocyte lysates from these patients. On two-dimensional gels, arginase protein was detected in any control subject, but it was completely absent in all the patients studied. These results suggest that either arginase protein in erythrocytes is not produced or it is structurally labile in these patients.
- Published
- 1991
- Full Text
- View/download PDF