Effect of ammonium chloride on homocitrulline and homoarginine synthesis from lysine

Kato, Tomoaki; Sano, M.

doi:10.1007/bf00714294

Cited by 6 publications

(2 citation statements)

References 2 publications

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“…The elevated homoarginine levels we measured in the ASSdeficient patient, were also reported in several other urea-cycle disorders [17,33]. Kato suggested that this elevation is the result of decreased degradation of lysine, increasing its availability as substrate for AGAT-catalyzed homoarginine synthesis [34]. Another possibility is downregulation of arginase as a compensatory reaction to the very low levels of arginine associated with ASS-deficiency.…”

Section: Discussionsupporting

confidence: 76%

Promiscuous activity of arginine:glycine amidinotransferase is responsible for the synthesis of the novel cardiovascular risk factor homoarginine

et al. 2012

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a b s t r a c tLow plasma homoarginine has emerged as a risk marker for cardiovascular disease. We exploited cells of a patient with a rare inborn error of metabolism to explore potential pathways of homoarginine synthesis, using stable isotopes and mass spectrometry. Control lymphoblasts, as opposed to lymphoblasts from an arginine:glycine amidinotransferase (AGAT)-deficient patient, were able to synthesize homoarginine from arginine and lysine. In contrast, in a patient with a deficiency of the urea cycle enzyme argininosuccinate synthase, plasma homoarginine was not decreased. We conclude that promiscuous activity of AGAT, a key enzyme in creatine synthesis, plays a pivotal role in homoarginine synthesis.

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Section: Discussionsupporting

confidence: 76%

Promiscuous activity of arginine:glycine amidinotransferase is responsible for the synthesis of the novel cardiovascular risk factor homoarginine

et al. 2012

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“…For this purpose, use of labelled homoarginine as a metaprobe is proposed (Fig. 4); this amino acid is present in detectable levels in fasting serum drawn from a number of mammalian species (Marescau et al 1992), including human subjects (Marescau et al 1995), and it appears to be produced as a minor metabolic consequence of hyperammonaemia (Kato et al 1988(Kato et al , 1989Kato & Sano, 1993). In addition, L-homoarginine is oxidized by both purified neuronal and macrophage NO synthase (I and II), with major formation of NO and homo-L-citrulline in a molar ratio of approximately 1 (Moali et al 1998).…”

Section: Some Novel Applications Of the Metaprobe Conceptmentioning

confidence: 99%

Metabolism 2000: the emperor needs new clothes

Young

Ajami

2001

Proc. Nutr. Soc.

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Professor Vernon R. Young, fax +1 617 253 9658, email vryoung@mit.edu Metabolism is one of the corner stones of nutritional science. As biology enters the post-genomic era and with functional genomics beginning to take off, we anticipate that the study of metabolism will play an increasingly important role in helping to link advances made via the reductionist paradigm, that has been so successful in molecular and cellular biology, with those emerging from observational studies in animals and human subjects. A reconstructive metabolically-focused approach offers a timely paradigm for enhancing the elegance of nutritional science. Here we give particular attention to the use of tracers as phenotyping tools and discuss the application of our metaprobe concepts with respect to some novel features of metabolism, including 'underground metabolism', 'metabolic hijacking', 'catalytic promiscuity' and 'moonlighting proteins'. The opportunities for enhancing the study of metabolism by new and emerging technologies, and the importance of the interdisciplinary research enterprise are also touched upon. We conclude that: (1) the metaprobe concepts and approach, discussed herein, potentially yield a quantitative physiological (metabolic) phenotype against which to elaborate partial or focused genotypes; (2) physiological (metabolic) phenotypes which have a whole-body or kinetically-discernible inter-organ tissue-directed metabolic signature are an ideal target for this directed tracer-based definition of the 'functional' genotype; (3) metabolism, probed with tracer tool kits suitable for measuring rates of turnover, change and conversion, becomes in the current sociology of the 'Net', like AOL, Yahoo, Alta Vista, Lycos or Ask Jeeves, the portal for an exploration of the metabolic characteristics of the 'Genomics Internet'. Metabolism: Metaprobe: Tracers: Physiological (metabolic) phenotypes GSH, glutathione. ' "But he hasn't got anything on" a little child said.'

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Clinical and biochemical characteristics of patients with ornithine transcarbamylase deficiency

Peng

Mei

et al. 2020

Clinical Biochemistry

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Effect of ammonium chloride on homocitrulline and homoarginine synthesis from lysine

Cited by 6 publications

References 2 publications

Promiscuous activity of arginine:glycine amidinotransferase is responsible for the synthesis of the novel cardiovascular risk factor homoarginine

Promiscuous activity of arginine:glycine amidinotransferase is responsible for the synthesis of the novel cardiovascular risk factor homoarginine

Metabolism 2000: the emperor needs new clothes

Clinical and biochemical characteristics of patients with ornithine transcarbamylase deficiency

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