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Gene Review:

Pycr1 - pyrroline-5-carboxylate reductase 1

Mus musculus

Synonyms: P5C reductase 1, P5CR 1, Pyrroline-5-carboxylate reductase 1, mitochondrial

Matsuzawa, T., Shiono, T. et al., Deutch, C.E. et al.

Deutch, Klarstrom, Link, Ricciardi,

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Disease relevance of Pycr1

E. coli strains with greatly reduced P5C reductase activity due to a proC mutation had no detectable T4C dehydrogenase activity [1].
In C3H retinal degeneration mice, P5C reductase activity in the retina and choroid was decreased to about one-third that of CRJ control mice in the specific activity after the tenth day [2].

High impact information on Pycr1

Addition of proline, the reduction product of P5C, did not result in an increase, suggesting that stimulation of the HMP pathway is related to the reduction of P5C to proline by the enzyme P5C reductase [3].
Stimulation of the hexose monophosphate pathway by pyrroline-5-carboxylate reductase in the lens [3].
Although P5C reductase did not act on proline, it also catalyzed the oxidation of 3,4-dehydroproline [1].
Postnatal changes of P5C reductase and ornithine oxoacid aminotransferase activities in the livers of C3H mice showed a pattern that reflected a delayed weaning [2].

References

Oxidation of L-thiazolidine-4-carboxylate by delta1-pyrroline-5-carboxylate reductase in Escherichia coli. Deutch, C.E., Klarstrom, J.L., Link, C.L., Ricciardi, D.L. Curr. Microbiol. (2001) [Pubmed]
Disease of the ornithine-proline pathway: delta 1-pyrroline-5-carboxylate reductase deficiency in the retina of retinal degeneration mice. Matsuzawa, T. Metabolic, pediatric, and systemic ophthalmology. (1982) [Pubmed]
Stimulation of the hexose monophosphate pathway by pyrroline-5-carboxylate reductase in the lens. Shiono, T., Kador, P.F., Kinoshita, J.H. Exp. Eye Res. (1985) [Pubmed]

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