1997
DOI: 10.1007/bf01372588
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The proline biosynthesis in living organisms

Abstract: Summary.In this article we review recent work on the physiology of proline and Al-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 … Show more

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Cited by 35 publications
(27 citation statements)
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“…Although it is often stated that proline oxidase is present mainly in the liver, kidney and brain [48,84] and that proline is not catabolized in the gut [47,85], relatively high activities of proline oxidase have been found in the small intestine and enterocytes of pigs [82,86] and rats [82]. In fact, the activity of proline oxidase in the pig small intestine is several-fold greater than that in the liver and kidney [42,86].…”
Section: Intestinal Arginine Synthesismentioning
confidence: 99%
“…Although it is often stated that proline oxidase is present mainly in the liver, kidney and brain [48,84] and that proline is not catabolized in the gut [47,85], relatively high activities of proline oxidase have been found in the small intestine and enterocytes of pigs [82,86] and rats [82]. In fact, the activity of proline oxidase in the pig small intestine is several-fold greater than that in the liver and kidney [42,86].…”
Section: Intestinal Arginine Synthesismentioning
confidence: 99%
“…In intact plant mitochondria fed with ornithine very little release of P5C was detected, supporting the concept that glutamate is an obligate intermediate in the conversion of ornithine to proline in plants (Elthon and Stewart 1982). The situation seems to be different in mammalian cells, where dOAT, P5CS and at least one P5CR isoform might colocalize in mitochondria (Aral and Kamoun 1997;Hu et al 2008b;Reversade et al 2009). In humans, dOAT deficiency is associated with high ornithine levels in blood and urine (Ramesh et al 1991).…”
Section: Proline Biosynthesismentioning
confidence: 99%
“…1). Acetylation acts as a protecting group strategy, preventing the spontaneous cyclization of the glutamate semialdehyde intermediate, thus precluding the formation of the proline precursor pyrroline-5-carboxylate (4). Two alternative pathways have evolved for the subsequent removal of the acetyl group and regulation of metabolic flow through the pathway.…”
mentioning
confidence: 99%