European Journal of Pediatrics

, Volume 150, Issue 11, pp 800–803 | Cite as

Absence of erythrocyte arginase protein in Japanese patients with hyperargininemia

  • C. Hayakawa
  • S. Aono
  • H. Keino
  • N. Mizutani
  • K. Watanabe
  • M. Ikemoto
  • M. Totani
  • T. Murachi
  • S. Kashiwamata
Metabolic Diseases
  • 19 Downloads

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.

Key words

Arginase Hyperargininemia Hyperammonemia 

Abbreviations

SDS

sodium dodecyl sulfate

PAGE

polyacrylamide gel electrophoresis

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Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • C. Hayakawa
    • 1
  • S. Aono
    • 2
  • H. Keino
    • 2
  • N. Mizutani
    • 1
  • K. Watanabe
    • 1
  • M. Ikemoto
    • 3
  • M. Totani
    • 4
    • 5
  • T. Murachi
    • 5
  • S. Kashiwamata
    • 2
  1. 1.Department of PediatricsNagoya University School of MedicineNagoya 466Japan
  2. 2.Department of PerinatologyInstitute for Developmental Research, Aichi Prefecture ColonyKasugai, AichiJapan
  3. 3.College of Medical TechnologyKyoto UniversityKyoto 606Japan
  4. 4.The National Institute of Health and NutritionTokyo 162Japan
  5. 5.Department of Clinical Science and Laboratory MedicineKyoto University Faculty of MedicineKyoto 606Japan

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