Absence of erythrocyte arginase protein in Japanese patients with hyperargininemia
| Autor: | Takashi Murachi, H. Keino, Shigeo Kashiwamata, S. Aono, Naoki Mizutani, Masayuki Totani, C. Hayakawa, Kimi Watanabe, Masaki Ikemoto |
|---|---|
| Rok vydání: | 1991 |
| Předmět: |
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 |
| Zdroj: | European Journal of Pediatrics. 150:800-803 |
| ISSN: | 1432-1076 0340-6199 |
| DOI: | 10.1007/bf02026716 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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