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Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia

The Journal of clinical investigation, 1979-12, Vol.64 (6), p.1544-1551

1979

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Protective effects of SR 57746A in central and peripheral models of neurodegenerative disorders in rodents and primates

Neuroscience, 1993-08, Vol.55 (3), p.629-641

1993

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Inhibition of ureagenesis by valproate in rat hepatocytes. Role of N-acetylglutamate and acetyl-CoA

Biochemical journal, 1983-10, Vol.216 (1), p.233-236

1983

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Role of N-acetylglutamate and acetyl-CoA in the inhibition of ureagenesis by isovaleric acid in isolated rat hepatocytes

Biochimica et biophysica acta, 1983-11, Vol.761 (1), p.13-16

1983

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Effect of overnight constant infusion of human growth hormone (GH)-releasing hormone-(1-44) on 24-hour GH secretion in children with partial GH deficiency

The journal of clinical endocrinology and metabolism, 1986-11, Vol.63 (5), p.1100-1105

1986

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N-Acetyl glutamate synthetase in human liver: regulation of activity by L-arginine and N-acetylglutamate

Biochemical and biophysical research communications, 1981-01, Vol.102 (3), p.1016-1020

1981

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Growth hormone-releasing factor modifies dopaminergic but not serotonergic activity in the arcuate nucleus of hypothalamus in the rat, as recorded in vivo by differential pulse voltammetry

Brain research, 1985-12, Vol.348 (2), p.367-370

1985

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A new family affected by the syndrome of hyperornithinaemia, hyperammonaemia and homocitrullinuria

Journal of inherited metabolic disease, 1987-03, Vol.10 (1), p.73-81

1987

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Potentiation by ammonia of the metabolic effects of pent-4-enoate in isolated rat hepatocytes

Biochemical journal, 1984-11, Vol.224 (1), p.263-267

1984

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Modified colorimetry of free fatty acids in plasma in the presence of valproic acid

Clinical chemistry (Baltimore, Md.), 1985-06, Vol.31 (6), p.1101-1101

1985

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Neonatal glutaric aciduria type II: an X-linked recessive inherited disorder

Human genetics, 1981-01, Vol.59 (3), p.263-265

1981

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CONGENITAL LACTIC ACIDOSIS, α‐KETOGLUTARIC ACIDURIA AND VARIANT FORM OF MAPLE SYRUP URINE DISEASE DUE TO A SINGLE ENZYME DEFECT: DIHYDROLIPOYL DEHYDROGENASE DEFICIENCY

Acta Paediatrica, 1982-01, Vol.71 (1), p.167-171

1982

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Occurrence of hyperammonemia in the course of 17 cases of methylmalonic acidemia

The Journal of pediatrics, 1981-08, Vol.99 (2), p.279-280

1981

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Results of 1-year growth hormone (GH)-releasing hormone-(1-44) treatment on growth, somatomedin-C, and 24-hour GH secretion in six children with partial GH deficiency

The journal of clinical endocrinology and metabolism, 1987-08, Vol.65 (2), p.268-274

1987

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Dichloroacetate as treatment for congenital lactic acidosis

The New England journal of medicine, 1978-12, Vol.299 (24), p.1365-1366

1978

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Acute glucagon treatment in rats fed various protein diets effect on n-acetyl glutamate concentration

Advances in experimental medicine and biology, 1982, Vol.153, p.217

1982

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Potentiation by piridoxilate of the synthesis of hippurate from benzoate in isolated rat hepatocytes. An approach to the determination of new pathways of nitrogen excretion in inborn errors of urea synthesis

Clinica chimica acta, 1984-01, Vol.136 (2), p.211-217

1984

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Letter to the Editor: A Mechanism for Valproate-Induced Hyperammonemia

Pediatric research, 1981-06, Vol.15 (6), p.974-975

1981

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Secondary citrullinemia with hyperammonemia in four neonatal cases of pyruvate carboxylase deficiency

Pediatrics (Evanston), 1981-12, Vol.68 (6), p.914-914

1981

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DEFECTIVE BIOTIN ABSORPTION IN MULTIPLE CARBOXYLASE DEFICIENCY

The Lancet (British edition), 1981-08, Vol.318 (8240), p.263-263

1981

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Discontinuity in the fall of left-handedness in a French population: A May '68 effect?

Laterality (Hove), 2006-01, Vol.11 (1), p.33-35

2006

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Inhibition of urea synthesis by pent-4-enoic acid: Potentiation by ammonia

Biochemical and biophysical research communications, 1984-01, Vol.118 (1), p.47-52

1984

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A mechanism for valproate-induced hyperammonemia

Pediatric research, 1981-06, Vol.15 (6), p.974

1981

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Correlation between blood ammonia concentration and organic acid accumulation in isovaleric and propionic acidemia

Pediatrics (Evanston), 1982-01, Vol.69 (1), p.115

1982

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Neonatal management of organic acidurias: clinical update

Journal of inherited metabolic disease, 1984, Vol.7, p.2-9

1984

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Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia

Protective effects of SR 57746A in central and peripheral models of neurodegenerative disorders in rodents and primates

Inhibition of ureagenesis by valproate in rat hepatocytes. Role of N-acetylglutamate and acetyl-CoA

Role of N-acetylglutamate and acetyl-CoA in the inhibition of ureagenesis by isovaleric acid in isolated rat hepatocytes

Effect of overnight constant infusion of human growth hormone (GH)-releasing hormone-(1-44) on 24-hour GH secretion in children with partial GH deficiency

N-Acetyl glutamate synthetase in human liver: regulation of activity by L-arginine and N-acetylglutamate

Growth hormone-releasing factor modifies dopaminergic but not serotonergic activity in the arcuate nucleus of hypothalamus in the rat, as recorded in vivo by differential pulse voltammetry

A new family affected by the syndrome of hyperornithinaemia, hyperammonaemia and homocitrullinuria

Potentiation by ammonia of the metabolic effects of pent-4-enoate in isolated rat hepatocytes

Modified colorimetry of free fatty acids in plasma in the presence of valproic acid

Neonatal glutaric aciduria type II: an X-linked recessive inherited disorder

CONGENITAL LACTIC ACIDOSIS, α‐KETOGLUTARIC ACIDURIA AND VARIANT FORM OF MAPLE SYRUP URINE DISEASE DUE TO A SINGLE ENZYME DEFECT: DIHYDROLIPOYL DEHYDROGENASE DEFICIENCY

Occurrence of hyperammonemia in the course of 17 cases of methylmalonic acidemia

Results of 1-year growth hormone (GH)-releasing hormone-(1-44) treatment on growth, somatomedin-C, and 24-hour GH secretion in six children with partial GH deficiency

Dichloroacetate as treatment for congenital lactic acidosis

Acute glucagon treatment in rats fed various protein diets effect on n-acetyl glutamate concentration

Potentiation by piridoxilate of the synthesis of hippurate from benzoate in isolated rat hepatocytes. An approach to the determination of new pathways of nitrogen excretion in inborn errors of urea synthesis

Letter to the Editor: A Mechanism for Valproate-Induced Hyperammonemia

Secondary citrullinemia with hyperammonemia in four neonatal cases of pyruvate carboxylase deficiency

DEFECTIVE BIOTIN ABSORPTION IN MULTIPLE CARBOXYLASE DEFICIENCY

Discontinuity in the fall of left-handedness in a French population: A May '68 effect?

Inhibition of urea synthesis by pent-4-enoic acid: Potentiation by ammonia

A mechanism for valproate-induced hyperammonemia

Correlation between blood ammonia concentration and organic acid accumulation in isovaleric and propionic acidemia

Neonatal management of organic acidurias: clinical update

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