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Biomedical subjects

L Cathelineau

Publications and source records attributed to L Cathelineau.

At least 37 records · Page 2Linked to original sources
PubMed1983

[Hyperammonemia during total parenteral nutrition in children].

Twenty-one children presenting with severe malnutrition were fed with total parenteral nutrition using 2 different aminoacids solutes containing 19% and 42% branched chain aminoacids respectively. With the 42% branched chain aminoacids solute a dysregulation of ammonia metabolism and changes in protein synthesis were observed. Several factors probably accounted for these changes. An excessive intake of branched chain aminoacids in children with malnutrition, under parenteral nutrition, is therefore iatrogenic and inappropriate.

Amino Acids, Branched-Chain↗
PubMed1982

Acute effects of glucagon on citrulline biosynthesis.

Mitochondria isolated from livers of rats fed on different diets showed altered capacity to synthesize citrulline. Glucagon, 15 min after injection, increases citrulline biosynthesis, except after the high-protein diet. A significant correlation between citrulline biosynthesis and N-acetylglutamate content with and without glucagon treatment was shown when rats were fed on a standard or a carbohydrate diet. Different diets modified carbamoyl phosphate synthetase I (EC 6.3.4.16) and N-acetylglutamate synthase (acetyl-CoA:L-glutamate N-acetyltransferase, EC 2.3.1.1) activities. Glucagon did not modify these activities.

Animals↗
PubMed1982

Ornithine transcarbamylase deficiencies in human males. Kinetic and immunochemical classification.

Enzymatic activities and kinetics of liver ornithine transcarbamylase (carbamoylphosphate:L-ornithine carbamoyltransferase) were studied in 16 human males with ornithine transcarbamylase mutations. In the same liver fragments, cross-reactive material was measured with specific anti-ornithine transcarbamylase antibody. These studies allowed us to describe five groups of mutations. Two of them were similar on the basis of their enzymatic properties and cross-reactive material amounts to two mouse ornithine transcarbamylase mutations:sparse-fur (spf) and sparse-fur with abnormal skin and hair (spf-ash).

Ammonia↗
PubMed1981

Studies on complementation in argininosuccinate synthetase and argininosuccinate lyase deficiencies in human fibroblasts.

Complementation tests after polyethylene glycol fusion have been performed between 10 citrullinemic strains with argininosuccinate synthetase deficiency and between five strains with argininosuccinate lyase deficiency. No complementation was observed between the citrullinemic strains, while two groups of complementation were defined with the argininosuccinate lyase deficients strains. Since the restoration of activity was not total, we assumed that complementation is probably intragenic.

Amino Acid Metabolism, Inborn Errors↗
PubMed1981

Renal Histidinuria.

One patient with abnormal histidinuria and a normal plasma histidine concentration is presented. This patient had myoclonic seizures like another patient previously reported with histidinuria. The effect of a peroral histidine loading test was studied. The relationship between renal histidinuria and neurological abnormalities is discussed.

Amino Acid Metabolism, Inborn Errors↗
PubMed1981

[Lysinuric protein intolerance: a severe hyperammonemia secondary to l-arginine deficiency (author's transl)]].

Lysinuric protein intolerance is an autosomal recessive disease, due to a defect in intestinal, renal and hepatic dibasic amino acid transport. Two new cases in the same family are reported. The disease appears progressively during the first months of life with failure to thrive, anorexia, vomiting, diarrhea, hepatosplenomegaly, muscular weakness, osteoporosis, anemia, leukothrombocytopenia, hyperammonemia and orotic aciduria after a high-protein intake. Hyperdibasicamino-aciduria was associated with subnormal plasma concentrations of the same aminoacids. Oral l-arginine, l-ornithine, l-lysine, and lysyl-glycine loads confirmed the diagnosis. The supplementation of the diet with l-citrulline resulted in normal levels of blood ammonia. However, hepatosplenomegaly, muscular weakness, osteoporosis remained unchanged and growth was not improved. These may be due to lysine deficiency.

Amino Acid Metabolism, Inborn Errors↗