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Activity of orotate metabolizing enzyme complex and various urea-cycle enzymes in mutant mice with ornithine transcarbamylase deficiency

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Summary

The overall activity of the enzyme complex consisting of orotate phosphoribosyl transferase and orotidine monophosphate decarboxylase, and of various enzymes of the urea cycle, has been studied in sparse-fur (spf) mutant mice with ornithine transcarbamylase deficiency. The enzyme complex has a lower overall activity, which could be caused by disturbed pyrimidine metabolism due to hyperammonemia. Other enzymes of the urea cycle do not show any significant change.

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Authors and Affiliations

  1. Laboratoire d'Endocrinologie, Métabolisme et Nutrition, Centre de Recherche Pédiatrique, Hôpital Sainte-Justine, 3175 Chemin Ste-Catherine, H3T 1C5, Montréal, Qué., Canada

    I. A. Qureshi, J. Letarte & R. Ouellet

Authors
  1. I. A. Qureshi
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  2. J. Letarte
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  3. R. Ouellet
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Additional information

Acknowledgments. The research was supported by a grant from the Conseil des Clubs Sociaux and Fondation Justine Lacoste Beaubien, Montréal. The authors wish to thank Ms Louise Poulin and Ms Francine Poisson for technical assistance.

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Qureshi, I.A., Letarte, J. & Ouellet, R. Activity of orotate metabolizing enzyme complex and various urea-cycle enzymes in mutant mice with ornithine transcarbamylase deficiency. Experientia 38, 308–309 (1982). https://doi.org/10.1007/BF01949358

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