Waste Nitrogen Excretion Via Amino Acid Acylation: Benzoate and Phenylacetate in Lysinuric Protein Intolerance

Treatment of Inborn Errors of Urea Synthesis

1982 • 266 citations

Treatment of Episodic Hyperammonemia in Children with Inborn Errors of Urea Synthesis

1984 • 230 citations

NEW PATHWAYS OF NITROGEN EXCRETION IN INBORN ERRORS OF UREA SYNTHESIS

1979 • 177 citations

Amino Acid Acylation: A Mechanism of Nitrogen Excretion in Inborn Errors of Urea Synthesis

1980 • 131 citations

Arginine, an indispensable amino acid for patients with inborn errors of urea synthesis.

1984 • 115 citations

Lysinuric protein intolerance: A two-year trial of dietary supplementation therapy with citrulline and lysine

1980 • 72 citations

Urinary metabolites characteristic of urea-cycle amino acid deficiency

1975 • 71 citations

Lysine Fluxes across the Jejunal Epithelium in Lysinuric Protein Intolerance

1980 • 67 citations

The Effect of Ammonia Administration on Orotic Acid Excretion in Rats

1965 • 64 citations

Rapid Determination of Ammonia in a Perchloric Acid Supernate from Blood, by Use of an Ammonia-Specific Electrode

1973 • 47 citations

Orotic Aciduria in Lysinuric Protein Intolerance: Dependence on the Urea Cycle Intermediates

1981 • 34 citations

SYNTHESIS OF AMINO ACIDS IN ANIMAL ORGANISMS. I. SYNTHESIS OF GLYCOCOLL AND GLUTAMINE IN THE HUMAN ORGANISM

1922 • 34 citations

“BASOLATERAL” AND MITOCHONDRIAL MEMBRANE TRANSPORT DEFECT IN THE HEPATOCYTES IN LYSINURIC PROTEIN INTOLERANCE

1983 • 23 citations

803 IDENTIFICATION OF HETEROZYGOSITY FOR ORNITHINE TRANSCARBAMYLASE DEFICIENCY (OTCD)

1985 • 9 citations