Skip to main content
SpringerLink
Log in

Glucose-6-P-Dehydrogenase Typ Bodensee (eine neue Enzymvariante)

Glucose-6-P-dehydrogenase “type bodensee” (a new enzyme variant)

  • Originalien
  • Published:
Klinische Wochenschrift Aims and scope Submit manuscript

Summary

A 22-year old patient with glucose-6-phosphate dehydrogenase deficiency and drug-induced hemolytic anemia was studied. Biochemical examination of the rough purified enzyme preparation of the erythrocytes showed a markedly increased turnover of substrate analogues and little thermal stability. The electrophoretic migration velocity, the Michaelis constants of glucose-6-phosphate and NADP as well as the pH activity of the enzyme were normal. It is recommended that this variant be termed the “type Bodensee” variant.

Zusammenfassung

Wir untersuchten einen 22jährigen Patienten mit Glucose-6-P-Dehydrogenase-Mangel und einer medikamenten-induzierten hämolytischen Anämie. Die biochemische Untersuchung der angereicherten Enzympräparation aus den Erythrocyten ergab einen stark erhöhten Umsatz von Substratanalogen und eine geringe Hitzestabilität. Die elektrophoretische Wanderungsgeschwindigkeit, die Michaeliskonstanten für G-6-P und NADP sowie das pH-Aktivitätsverhalten des Enzyms waren normal. Wir schlagen für diese Variante den Namen Typ Bodensee vor.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Literatur

  1. Beisenherz, G., Boltze, H. J., Bücher, Th., Czok, R., Garbade, K. H., Meyer-Arendt, E., Pfleiderer, G.: Diphospho-Fructose-Aldolase, Phosphoglyceraldehyd-Dedrogenase, Milchsäure-Dehydrogenase, Glycerophosphat-Dehydrogenase und Pyruvatkinase aus Kaninchenmuskulatur in einem Arbeitsgang. Z. Naturforsch.8b, 555–577 (1953).

    Google Scholar 

  2. Benöhr, H. Chr., Waller, H. D.: Eigenschaften der Glucose-6-P-Dehydrogenase Typ Tübingen. Klin. Wschr.48, 71–74 (1970).

    Google Scholar 

  3. —— Usener, H., Wagner, H., Kleihauer, E., Waller, H. D.: Spontane Hämolyse bei einem Kranken mit Glukose-6-P-Dehydrogenase-Mangel (mediterraner Typ) und heterozygoter Beta-Thalassämie. Med. Welt21, 1091–1095 (1970).

    Google Scholar 

  4. Beutler, E.: Genetic variability of G-6-PD. Hum. Genet.9, 250–252 (1970).

    Google Scholar 

  5. —— Drug-induced hemolytic anemia. Pharmacol. Rev.21, 73–103 (1969).

    Google Scholar 

  6. —— Dern, R. J., Alving, A. S.: The hemolytic effect of primaquine. III. A study of primaquine-sensitive erythrocytes. J. Lab. clin. Med.44, 177–184 (1954).

    Google Scholar 

  7. —— Duron, O., Kelly, B.: Improved method for the determination of blood glutathione. J. Lab. clin. Med.61, 882–888 (1963).

    Google Scholar 

  8. —— Mathai, C. K., Smith, J. E.: Biochemical variants of glucose-6-phosphate dehydrogenase giving rise to congenital nonspherocytic hemolytic disease. Blood31, 131–150 (1968).

    Google Scholar 

  9. Botha, M. C., Dern, R. J., Mitchell, M., West, C., Beutler, E.: G-6-PD Capetown, a new variant of glucose-6-phosphate dehydrogenase. Amer. J. hum. Genet.21, 547–551 (1969).

    Google Scholar 

  10. Bücher, Th.: Phosphoglycerate kinase from Brewers yeast. In: Methods in enzymology, vol. I, p. 415. New York-London: Acad. Press 1955.

    Google Scholar 

  11. Busch, D., Pelz, K.: Erythrozytenisolierung aus Blut mit Baumwolle. Klin. Wschr.44, 983–984 (1966).

    Google Scholar 

  12. Delbrück, A., Zebe, E., Bücher, Th.: Über die Verteilungsmuster von Enzymen des energieliefernden Stoffwechsels im Flugmuskel, Sprungmuskel und Fettkörper von Locusta migratoria und ihre cytologische Zuordnung. Biochem. Z.331, 273–296 (1959).

    Google Scholar 

  13. Grignani, F., Löhr, G. W.: Über die Hexokinase in menschlichen Blutzellen. Klin. Wschr.38, 796–801 (1960).

    Google Scholar 

  14. Horecker, B. L., Smyrniotis, P. Z.: 6-phosphogluconic dehydrogenase. In: Methods in enzymology, vol. I, p. 323–327. New York-London: Acad. Press 1955.

    Google Scholar 

  15. Kirkman, H. N., Brinson, G. A., Pickard, B. M.: New variants of glucose-6-phosphate dehydrogenase in caucasian males. Proc. 9. Congr. Europ. Soc. Haemat. 685–688. Lisbon 1963.

  16. —— Kidson, C., Kennedy, M.: Variants of human glucose-6-phosphate dehydrogenase. Studies of samples from New Guinea. In: Hereditary disorders of erythrocyte metabolism, p. 126–145. New York: Grune & Stratton 1968.

    Google Scholar 

  17. —— Schettini, F., Pickard, B. M.: Mediterranean variant of glucose-6-phosphate dehydrogenase. J. Lab. clin. Med.63, 726–738 (1964).

    Google Scholar 

  18. Kosower, N. S., Vanderhoff, G. A., London, I. M.: The regeneration of reduced glutathione in normal and glucose-6-phosphate dehydrogenase deficient human red blood cells. Blood29, 313–319 (1967).

    Google Scholar 

  19. Kubowitz, F., Ott, P.: Isolierung und Kristallisation eines Gärungsferments aus Tumoren. Biochem. Z.314, 94–117 (1943).

    Google Scholar 

  20. Löhr, G. W., Waller, H. D.: Glucose-6-Phosphat-Dehydrogenase (Zwischenferment). In: Methoden der enzymatischen Analyse, S. 744–751. Weinheim: Chemie-Verlag 1962.

    Google Scholar 

  21. Marks, P. A., Banks, J., Gross, R. T.: Genetic heterogeneity of glucose-6-phosphate dehydrogenase deficiency. Nature (Lond.)194, 454–456 (1962).

    Google Scholar 

  22. McCurdy: Personal communication. Zit. nach E. Beutler 5.

    Google Scholar 

  23. Motulsky, A. G., Kraut, J. M., Thieme, W. T., Musto, D. F.: Biochemical genetics of glucose-6-phosphate dehydrogenase deficiency. Clin. Res.7, 89 (1959).

    Google Scholar 

  24. — Diskussionsbemerkung zu E. Beutler 4.

  25. Racker, E.: Spectrophotometric measurement of hexokinase and phosphohexokinase activity. J. biol. Chem.167, 843–854 (1947).

    Google Scholar 

  26. —— Glutathione reductase (liver and yeast). In: Methods in enzymology, vol. II, p. 722–725. New York-London: Acad. Press 1955.

    Google Scholar 

  27. Ramot, B., Ben-Bussat, J., Schhory, M.: New glucose-6-phosphate dehydrogenase variants in Israel and their association with congenital nonspherocytic hemolytic disease. J. Lab. clin. Med.74, 895–901 (1969).

    Google Scholar 

  28. Ratazzi, M. C., Bernini, L. F., Fiorelli, G., Mannucci, P.: Electrophoresis of G-6-P-dehydrogenase. Nature (Lond.)213, 79–80 (1967).

    Google Scholar 

  29. Schröter, W., Kalinowsky, W.: Erythrocyte 2,3-diphosphoglycerate mutase: an optical test in hemolysates. Clin. chim. Acta25, 283–285 (1969).

    Google Scholar 

  30. Slein, M. W.: Phosphohexoisomerase from muscle. In: Methods in enzymology, vol. I, p. 299–306. New York-London: Acad. Press 1955.

    Google Scholar 

  31. Stamatoyannopoulos, G., Yoshida, A., Bacopoulos, C., Motulsky, A. G.: Athens variant of glucose-6-phosphate dehydrogenase. Science157, 831–833 (1967).

    Google Scholar 

  32. Sutherland, E. W., Posternak, T., Cori, C. F.: Mechanism of the phosphoglycerate mutase reaction. J. biol. Chem.181, 153–159 (1949).

    Google Scholar 

  33. Talalak, P., Beutler, E.: G-6-PD Bangkok: a new variant found in congenital nonspherocytic hemolytic disease. Blood33, 772–776 (1969).

    Google Scholar 

  34. Vogell, W., Bishay, F. R., Bücher, Th., Klingenberg, M., Pette, D., Zebe, E.: Über strukturelle und enzymatische Muster in Muskeln von Locusta migratoria. Biochem. Z.332, 81–117 (1959).

    Google Scholar 

  35. World Health Organization Technical Report Series: Standardization of procedures for the study of glucose-6-phosphate dehydrogenase. Report of a WHO scientific group. Wld Hlth Org. techn. Rep. Ser.366, 1967.

  36. Yoshida, A., Baur, E. W., Motulsky, A. G.: A philippine glucose-6-phosphate dehydrogenase variant (G6PD Union) with enzyme deficiency and altered substrate specifity. Blood35, 506–513 (1970).

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Medizinische Klinik, o. Lehrstuhl, und Abteilung Innere Medizin II der Universität Tübingen, Germany

    H. Chr Benöhr, H. D. Waller, H. Arnold, K. G. Blume & G. W. Löhr

  2. der Medizinischen Klinik der Universität Freiburg, Germany

    H. Chr Benöhr, H. D. Waller, H. Arnold, K. G. Blume & G. W. Löhr

Authors
  1. H. Chr Benöhr
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H. D. Waller
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. H. Arnold
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. K. G. Blume
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. G. W. Löhr
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Mit dankenswerter Unterstützung durch die Deutsche Forschungsgemeinschaft.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Benöhr, H.C., Waller, H.D., Arnold, H. et al. Glucose-6-P-Dehydrogenase Typ Bodensee (eine neue Enzymvariante). Klin Wochenschr 49, 1058–1062 (1971). https://doi.org/10.1007/BF01732914

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01732914

Key-words

Schlüsselwörter

Search

Navigation