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Journal of Inherited Metabolic Disease

, Volume 11, Issue 2, pp 166–173 | Cite as

Biochemical studies on prolidase in sera from control, patients with prolidase deficiency and their mother

  • T. Ohhashi
  • T. Ohno
  • J. Arata
  • H. Kodama
Article

Summary

Prolidase activity in serum from normal subjects and the mother of two patients was readily detected without adding Mn2+ to the assay, and the activity was increased by addition of Mn2+ to the assay or preincubation with Mn2+. However, the activity in serum from patients with prolidase deficiency against gly-pro, leu-pro and val-pro could not be detected irrespective of Mn2+ conditions and activity against met-pro, ala-pro and phe-pro also showed a marked reduction compared to controls. Both normal and the patients' mother's prolidase activity against gly-pro was reduced about 20% at 60°C compared to the activity at 37°C, but the addition of Mn2+ at 55°C increased the activity about 1.8-fold, whereas prolidase activity of patients could not be increased by the addition of Mn2+. The addition of Co2+ increased prolidase activity in serum from control and the patients' mother but did not increase the heat stability. These results indicate that prolidase in serum from patients with prolidase deficiency is altered rather than markedly reduced in amount.

Keywords

Public Health Internal Medicine Metabolic Disease Marked Reduction Biochemical Study 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Arata, J., Umemura, S., Yamamoto, Y., Hagiyama, M. and Nohara, N. Prolidase deficiency, its dermatological manifestations and same additional biochemical studies.Arch. Dermatol. 115 (1979) 62–68Google Scholar
  2. Butterworth, J. and Priestman, D. Substrate specificity of manganese-activated prolidase in control and prolidase-deficient cultured skin fibroblasts.J. Inher. Metab. Dis. 7 (1984) 32–34Google Scholar
  3. Charpentier, C., Dagbovie, K., and Lemonnier, A. Prolidase deficiency with iminopeptiduria; biochemical investigations and first results of attempted therapy.J. Inher. Metab. Dis. 4 (1981) 77–78Google Scholar
  4. Chinard, F. P. Photometric estimation of proline and ornithine.J. Biol. Chem. 199 (1952) 91–95Google Scholar
  5. Goodman, St. I., Salomons, C. C., Muschenheim, F., McIntyre, C. A., Miles, B. and O'Brien, D. A syndrome resembling lathyrism associated with iminodipeptiduria.Am. J. Med. 45 (1968) 152–160Google Scholar
  6. Hui, K. S. and Leita, A. Prolidase activity in brain comparison with other organs.J. Neurochem. 30 (1972) 1113–1123Google Scholar
  7. Kodama, H., Umemura, S., Shimomura, M., Mizuhara, S., Arata, J., Yamamoto, Y., Yasutake, A. and Izumiya, N. Studies on a patient with iminopeptiduria. 1. Identification of urinary iminopeptides.Physiol. Chem. Phys. 8 (1976) 463–473Google Scholar
  8. Myara, I., Charpentier, C. and Lemonnier, A. Optimal conditions for prolidase assay by proline colorimetric determination: Application to iminodipeptiduria.Clin. Chim. Acta 125 (1982) 193–205Google Scholar
  9. Powell, G. F., Rasco, M. A. and Maniscalo, R. M. A prolidase deficiency in man with iminodipeptiduria.Metabolism 23 (1974) 505–513Google Scholar
  10. Priestman, D. and Butterworth, J. Prolidase deficiency; Characteristics of human skin fibroblast prolidase using colorimetric and fluorimetric assays.Clin. Chim. Acta 16 (1984) 227–231Google Scholar
  11. Sheffield, L. J. Schlesinger, P., Faull, K., Halpern, B. J., Schier, G. M., Cotton, R. G. H., Hammond, J. and Danks, D. M. Iminopeptiduria, skin ulcerations, and edema in a boy with prolidase deficiency.J. Pediatrics 91 (1977) 578–583Google Scholar

Copyright information

© SSIEM and MTP Press Ltd 1988

Authors and Affiliations

  • T. Ohhashi
    • 1
  • T. Ohno
    • 2
  • J. Arata
    • 2
  • H. Kodama
    • 1
  1. 1.Department of ChemistryKochi Medical SchoolKochiJapan
  2. 2.Department of DermatologyKochi Medical SchoolKochiJapan

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