Advances in Experimental Medicine and Biology

New Facets in Urea Cycle Disorders.- Newborn screening for urea cycle disorders.- A new method for screening of hyperammonemia.- Immobilization of multienzymes of urea cyle into fibrin membrane: an approach to an artificial liver.- N-acetylglutamate synthetase (NAGS) deficiency: diagnosis, clinical observations and treatment.- Ornithine transcarbamylase (OTC) in white blood cells and jejunal mucosa.- Immunochemical assay in 16 boys with ornithine transcarbamylase deficiency.- Enzymatic analysis of citrullinemia (12 cases) in Japan.- Qualitative abnormality of liver argininosuccinate synthetase in a patient with citrullinemia.- Argininosuccinic aciduria in adult: a clinical, electrophysiological and biochemical study.- First case of argininosuccinic aciduria in Japan: clinical observations and treatment.- Complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts.- Clinical and biochemical findings in argininemia.- Argininemia: report of a new case and mechanisms of orotic aciduria and hyperammonemia.- Therapy of neonatal onset urea cycle enzymopathies (UCE).- to the Session on Secondary and Transient Hyperammonemia.- Hyperammonemia secondary to hereditary organic acidurias: a study of 29 cases.- Neonatal isovaleric acidemia associated with hyperammonemia.- Hyperammonemia in the neonate with hypoxia.- A mechanism for valproate-induced hyperammonemia.- Reduced activity of OTC in the liver of patient with Reye's syndrome.- Spontaneous animal models of ornithine transcarbamylase deficiency studies on serum and urinary nitrogenous metabolites.- Sparse-fur mutation: a model for some human orni-thine transcarbamylase deficiencies.- Regulation of the N-acetylglutamate content of rat hepatocytes by the glutamate concentration.- Enzyme regulation of N-acetylglutamate synthesis in mouse and rat liver.- Acute glucagon treatment in rats fed various protein diets. Effect on N-acetylglutamate concentration.- The relation between the developmental timing of birth and developmental increases in urea cycle enzymes.- Studies on the enzymes of urea cycle intermediates in normal and infarcted myocardial tissue of rat.- Effects of arginine-free meals on ureagenesis in cats.- Dynamism of rat liver ornithine metabolisms in relation to high-protein stimulation of the urea cycle.- Regulation of urea synthesis: changes in the concentration of ornithine in the liver corresponding to changes in urea synthesis.- Synthesis and intracellular transport of mitochondrial carbamylphosphate synthetase I and ornithine transcarbamylase.- Isolation of argininosuccinase from bovine brain: catalytic, physical and chemical properties compared to liver and kidney enzymes.- On the mechanism of the alterations of rat kidney transamidinase activities by diet and hormones.- Sciatectomic stimulation of muscle arginase and its implications.- Enzymes of arginine metabolism in the lizard, calotes versicolor.- Orotic acid in urine and hyperammonemia.- Comparison between amino acids and orotic acid analysis in the detection of urea cycle disorders in the Quebec urinary screening program.- Transient hyperammonemias in infants with and without organic academia.- Mechanism of hyperammonemia in an experimental model of propionic academia.- The study of organic acid metabolism in a patient with ornithine transcarbamylase (OTC) deficiency.- Hyperornithinemia, gyrate atrophy and ornithine keto-acid transaminase.- Gyrate atrophy of the choroid and retina (GA): toxic effects of ornithine and long-term therapy with an arginine-restricted diet.- Disease of ornithine-proline pathway: an ?'-pyrroline-5-carboxylate reductase deficiency in the retina of retinal degeneration mice.- Toxic effects of ornithine and its related compounds on the retina.- Quantitative determination of guanidino compounds: the excellent preparation of biological samples.- Recommended deproteinizing methods for plasma guanidino compounds analysis by liquid chromatography.- Evolutionary relationship between arginine and creatine in muscle.- Metabolism of arginine in invertebrates: relation to urea cycle and to other guanidine derivates.- a Guanidinoglutaric acid and epilepsy.- Guanidino compounds in hyperargininemia.- Guanidinosuccinic acid and the alternate urea cycle.- Guanidinosuccinic acid excretion in argininosuccinic aciduria.- Metabolic pathway of guanidino compounds in chronic renal failure.- Guanidino compounds and hemodialysis.- Effect of guanidino compounds on hen egg development.- a-ketoglutarate induced transamination during ischemic exercise.- Effect of pyridoxine-2-oxoglutarate administration in patients with advanced cirrhosis: control of ammonia pyruvate and lactate high plasma concentrations.- Treatment of pyruvic and lactic acidaemia in ophthalmoplegia plus.- Intracerebral pH regulation and ammonia detoxification.- Concluding Remarks.- Participants.