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The proline biosynthesis in living organisms

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Summary

In this article we review recent work on the physiology of proline and Δ1-pyrroline-5-carboxylate (P5C) in living organisms and consider recent progress in our understanding of the role of P5C synthetase in collagen metabolism and the regulation of urea cycle in vertebrates. Much of this recent progress has been made possible by advances in our knowledge of the enzymes and genes involved in proline biosynthesis in man. The availability of well characterized P5C synthetase deficiency in man has been an impetus for the cloning of the cDNA encoding for this enzyme from man and facilitated the establishment of the phenotype-genotype relationships in P5C synthetase deficiency in higher vertebrates.

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Abbreviations

GK:

γ-glutamyl kinase

GPR:

γ-glutamyl phosphate reductase

P5CR:

Δ 1-pyrroline-5-carboxylate reductase

GSA:

glutamic-γ-semialdehyde

P5C:

Δ1-pyrroline-5-carboxylate

P1 :

Inorganic phosphate

AMP, ADP, ATP:

Adenosine 5′-mono-, di-, triphosphate

NAD+, NADH:

nicotinamide adenine dinucleotide, and its reduced form

NADP+, NADPH:

nicotinamide adenine dinucleotide phosphate, and its reduced form; DEAF: diethylaminoethyle

OAT:

ornithine amino transferase; CHO: Chinese hamster ovary

IGF-1:

insulin-like growth factor-1

P5CDH:

pyrroline 5carboxylate dehydrogenase

IMP:

inosine 5′-monophosphate

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  1. Laboratoire de Biochimie Médicale B and Centre National de la Recherche Scientifique (CNRS) URA1335, Hôpital Necker-Enfant Malades, 149, Rue de Sèvres, F-75015, Paris, France

    B. Aral &  P. Kamoun

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Aral, B., Kamoun, P. The proline biosynthesis in living organisms. Amino Acids 13, 189–217 (1997). https://doi.org/10.1007/BF01372588

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