Biochemical, morphological and hybrid studies in hyperprolinemic mice

Y. S. Kanwar, C. A. Krakower, J. R. Manaligod, P. Justice, P. W. Wong

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

Hyperprolinemia, hyperprolinuria and hydroxyprolinuria were observed in PRO/Re mice. Hepatic proline oxidase activity in PRO/Re mice was markedly deficient. It was demonstrated that the deficiency of proline oxidase activity was not due to the presence of an inhibitor. The mutant enzyme in PRO/Re showed no difference in heat stability but had a poor affinity for the substrate, L proline as compared to normal enzymes. There was no significant proteinuria or hematuria in PRO/Re mice. Their serum protein and blood urea nitrogen were normal. Morphologic studies by light and electron microscopy demonstrated no abnormality in the renal tissues of PRO/Re up to 6 mth of age, suggesting that hyperprolinemia did not cause renal damage. Pedigree studies showed that F 1 generation (PRO/Re x CD 1) had approximately 50% of normal proline oxidase activity and significantly higher plasma proline. The distribution of hepatic proline oxidase activity in F 2 generation (F 1 x F 1) was characteristic of an autosomal recessive trait.

Original languageEnglish (US)
Pages (from-to)209-216
Number of pages8
JournalBiomedicine
Volume22
Issue number3
StatePublished - Dec 1 1975

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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    Kanwar, Y. S., Krakower, C. A., Manaligod, J. R., Justice, P., & Wong, P. W. (1975). Biochemical, morphological and hybrid studies in hyperprolinemic mice. Biomedicine, 22(3), 209-216.