Diminished Phenylketonuria in Phenylpyruvic Oligophrenia After Administration of L-Glutamine, L-Glutamate or L-Asparagine1

Meister, Alton; Udenfriend, Sidney; Bessman, Samuel P.

doi:10.1172/jci103317

Cited by 33 publications

(5 citation statements)

References 30 publications

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“…Other treatments known to affect the plasma concentrations of glycine or phenylalanine metabolites had no effect on the cerebral glycine content of hyperphenylalaninemic rats. Glutamate and benzoate reduced the concentration of plasma glycine of hyperglycinemic patients (Nyhan, 1967;Baumgartner et al, 1969;Krieger and Hart, 1974), and glutamate reduced the plasma phenylpyruvate concentration in a phenylketonuric (Meister et al, 1956). In the present work, glutamate did not diminish the content of brain glycine within the time span of the experiment ( Table 2), and benzoate was lethal to most of the animals at the dose tested.…”

Section: Relationship To Mental Returdationsupporting

confidence: 47%

Chronic Hyperphenylalaninemia Produces Cerebral Hyperglycinemia in Immature Rats

Dienel

1981

Journal of Neurochemistry

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The amino acid content of three tissues was measured in 10-day-old rats made hyperphenylalaninemic from age 3 to 10 days by daily injection of phenylalanine plus alpha-methylphenylalanine to inhibit phenylalanine hydroxylase (PAH). At 12 h after the last injection, the concentrations of alanine, valine, methionine, isoleucine, and leucine in the cerebral hemispheres were depressed by 25-50%, whereas that of glycine was elevated 2.3-fold. In the spinal cord, the levels of phosphoserine, methionine, and leucine were decreased by 40-50%, and those of serine and threonine increased by 50%. Tyrosine and phenylalanine concentrations were high in all tissues, 2-3 and 15-30 times normal, respectively; of the amino acids investigated, they were the only ones changed in the liver. Cerebral hyperglycinemia was also produced by chronic treatment with phenylalanine plus p-chlorophenylalanine to inhibit PAH, but not by acute (12 h) hyperphenylalaninemia. An increase in cerebral phosphoserine phosphatase activity was greater in rats treated with phenylalanine plus PAH inhibitor than with inhibitor alone. The content of brain glycine normally declines with age from birth to 15 days; this decrease was prevented by chronic hyperphenylalaninemia. Attempts to reduce the cerebral glycine content of the hyperphenylalaninemic rats were unsuccessful. However, one of the therapeutic protocols, methionine loading, may be useful because it increased the methionine and decreased the phenylalanine contents in the brain.

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Section: Relationship To Mental Returdationsupporting

confidence: 47%

Chronic Hyperphenylalaninemia Produces Cerebral Hyperglycinemia in Immature Rats

Dienel

1981

Journal of Neurochemistry

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“…1). It is well established that glutamine is absorbed intact from the gastrointestinal tract (25) and that in normal man plasma glutamine levels rise promptly after oral administration (26). In both our gouty and nongouty subjects the plasma glutamine increased from initial mean levels of approximately 0.45 ,umole/ml to equal mean levels in excess of 0.70 ,mole/ min at the end of I hr; at 1J hr the values were declining, and at 2k hr had almost returned to the initial levels.…”

Section: Resultsmentioning

confidence: 99%

Plasma and urinary amino acids in primary gout, with special reference to glutamine

Yu¹,

Adler²,

Bobrow³

et al. 1969

J. Clin. Invest.

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“…Besides additional supplementation of tyrosine, different alternative SAA treatments have been reported. Apart from the very early but unsuccessful ideas about glutamine supplementation [ 57 - 59 ], the following suggestions on SAA supplementation have been proposed.…”

Section: Resultsmentioning

confidence: 99%

Single amino acid supplementation in aminoacidopathies: a systematic review

Vliet

Derks

Rijn

et al. 2014

Orphanet J Rare Dis

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Aminoacidopathies are a group of rare and diverse disorders, caused by the deficiency of an enzyme or transporter involved in amino acid metabolism. For most aminoacidopathies, dietary management is the mainstay of treatment. Such treatment includes severe natural protein restriction, combined with protein substitution with all amino acids except the amino acids prior to the metabolic block and enriched with the amino acid that has become essential by the enzymatic defect. For some aminoacidopathies, supplementation of one or two amino acids, that have not become essential by the enzymatic defect, has been suggested. This so-called single amino acid supplementation can serve different treatment objectives, but evidence is limited. The aim of the present article is to provide a systematic review on the reasons for applications of single amino acid supplementation in aminoacidopathies treated with natural protein restriction and synthetic amino acid mixtures.

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Diminished Phenylketonuria in Phenylpyruvic Oligophrenia After Administration of L-Glutamine, L-Glutamate or L-Asparagine1

Cited by 33 publications

References 30 publications

Chronic Hyperphenylalaninemia Produces Cerebral Hyperglycinemia in Immature Rats

Chronic Hyperphenylalaninemia Produces Cerebral Hyperglycinemia in Immature Rats

Plasma and urinary amino acids in primary gout, with special reference to glutamine

Single amino acid supplementation in aminoacidopathies: a systematic review

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