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Biomedical subjects

N Moatti

Publications and source records attributed to N Moatti.

At least 127 records · Page 7Linked to original sources
PubMed1980

Glycine transport by cultured skin fibroblasts from a patient with isolated hyperglycinuria.

Glycine transport is studied in cultured skin fibroblasts from a patient with isolated hyperglycinuria and from five normal subjects. Fibroblasts from the patient take up glycine less well than do cell lines from controls. Kinetic studies are consistent with a single transport system in the patient's and controls' cell lines. Vmax value in the hyperglycinuric lines is normal, but the apparent affinity is always reduced as opposed to those of four different control lines separately tested. Statistical analysis shows significant difference between Km values.

Amino Acid Metabolism, Inborn Errors↗
PubMed1979

[Regulation of glycogen metabolism in the liver and hepatic glycogenosis due to phosphorylase system deficiency].

Glycogen synthesis and breakdown in the liver are tightly controlled through different mechanisms. The purpose of this review is to describe some properties of the enzymes involved in the glycogen metabolism and the sequence of events by which glucose, allosteric effectors and hormones control this metabolism in the liver. Clinical, genetic and biological aspects of the phosphorylase and the phosphorylase kinase deficiencies are examined. The enzymatic analysis of the haemolysates from the patients allows discrimination of these two types of glycogenosis.

Glycogen Storage Disease↗
PubMed1979

[Hereditary abnormalities of galactose metabolism: diagnosis and biochemical supervision (author's transl)].

The authors define the main stages of the biochemical study of hereditary abnormalities of galactose metabolism. They review laboratory examinations for detection, enzyme examinations which provide the diagnostic proof, further examinations which permit one to follow the course and efficacy of a galactose-free diet, the demonstration of genetic variants, the technics of antenatal diagnosis and routine neonatal detection.

Biological Assay↗
PubMed1979

Study of some enzymatic activities in human liver cell cultures.

Some enzymatic activities were studied in long ter cultures of human liver cells : glucose-6-phosphatase, U.D.P. glucuronosyltransferase, phenylalanine 4-hydroxylase and tyrosine aminotransferase. Only weak tyrosine aminotransferase activity has been found in 12 subcultures, and it has not been increased by addition of corticoids. This tyrosine aminotransferase activity has been measured at different passages of the culture. Our results are compared with those found in literature. The different reasons which could explain the absence of liver specific biochemical functions have been discussed.

Cell Survival↗
PubMed1978

Effect of cycloleucine on amino acid accumulation by human diploid fibroblasts.

Cycloleucine is a synthetic amino acid which produces, in vivo, biochemical abnoramlities comparable to those seen in human cystinuria-lysinuria. The effect of cycloleucine on intracellular accumulation of amino acids overlapping separate transport systems was studied using human diploid fibroblasts subcultures on glass coverslips. The data indicated that alpha-alanine, serine and proline accumulation was inhibited significantly by cycloleucine. The percentage of inhibition was approximately the same. Lysine was less affected by cycloleucine, but this amino acid accumulation proceeded at a rate slower than for neutral amino acids. In vitro, this inhibitory effect seems to be a generalized phenomenon affecting substrates. These results confirm in human fibroblasts data reported for human and rat kidney slices.

Amino Acids↗
PubMed1977

Effects of serum-free culture media on human liver and fibroblastic cells.

The morphological aspects and amino acid variations of human fibroblast and liver cell monolayer cultures were studied in serum free media. Under these conditions, the behaviour of the two cell types differed greatly. The morphological changes for the liver cells, as compared with the fibroblasts, appear more quickly and some of these changes are particular. Furthermore, extracellular amino acid variations are of lesser importance for the liver cells, during the 10 days following the suppression of serum, except for serine.

Amino Acids↗
PubMed1976

[Effect of cycloleucine on renal uptake of dibasic amino acids and cystine].

Cycloleucinead ministration (a synthetic amino acid) to rats produces a selective hyperaminoaciduria bearing on dibasic amino acids (lysine, arginine, ornithine) and cystine. Interference of cycloleucine with tubular reabsorption of these amino acids was studied "in vitro", using rat kidney cortex slices. When dibasic amino acids and cystine are at physiological concentrations, the data indicate that cycloleucine decreases their intracellular accumulation. However, effect of cycloleucine "in vitro" is not specific whatsoever its concentration; similar results are obtained with neutral amino acids such as alpha-alanine and valine. Inhibitory effects are not modified by the presence of several amino acids in the incubation medium. The results are interpreted as evidence for a dissociation between "in vitro" cellular accumu-lation and "in vivo" transepithelial transport.

Amino Acids↗
PubMed1976

Comparative study of extracellular amino acids in culture of human liver and fibroblastic cells.

Amino acid concentration are studied in the extracellular media of ten series of human fibroblast and liver cell monolayer cultures. These two cell types consume and produce ostensively the same amino acids. Among the nonessential amino acids, the most significant variations involve serine and aspartate which are decreased; alpha-alanine, glutamate, ornithine and proline are, on the contrary, increased. Among the essential amino acids, leucine, isoleucine and glutamine are preferentially decreased. The variations of some amino acids are correlated with the cell density. The interrelations which may exist between the variations of these different amino acids are discussed. Furthermore, the glycolytic acitivity of the cells studied is very high: 85% of glucose consumed is found in the form of lactate.

Alanine↗
PubMed1975

[Fructose 1,6-diphosphatase deficiency in 2 sisters].

The discovery of a fructose-1,6-diphosphatase deficiency in two sisters leads to the discussion of the various loading tests which are required for the diagnosis. The diagnosis may be discussed clinically with type I glycogenosis, and biologically with hereditary fructose intolerance. The specific characteristics of these disorders are analyzed as well as the problem of fructose induced hypoglucosemia. The failure of the treatment with folic acid in one of the cases leads to emphasize the suppression of prolonged fast in order to avoid acute accidents.

Carbohydrate Metabolism, Inborn Errors↗