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Biomedical subjects

R Rothman

Publications and source records attributed to R Rothman.

27 records · Page 2Linked to original sources

Further evidence for an opioid receptor complex.

We recently presented evidence that distinct morphine and enkephalin receptors coexist in an opioid receptor complex. These studies used membranes prepared from whole rat brain. In this paper the receptor complex is demonstrated to occur in membranes prepared from rat striatum, cortex, and pooled nonstriatal-noncortical regions of the brain. The observation that morphine masks enkephalin receptors is confirmed using 3H-methionine enkephalin to label the enkephalin receptor. These data further support the hypothesis that populations of morphine and enkephalin receptors coexist in an opioid receptor complex.

Animals↗

Holocarboxylase synthetase deficiency: a biotin-responsive organic acidemia.

The clinical and biochemical features of an infant affected by holocarboxylase synthetase deficiency are presented. The patient was the sibling of the deceased child in whose cultured skin fibroblasts the precise enzymatic disorder was first determined. This fact permitted administration of specific therapy in the form of oral biotin, resulting in immediate improvement from impending respiratory failure and shock. The clinical response to biotin was accompanied by recovery of the biochemical mechanisms known to be biotin-dependent, as manifested by disappearance of intermediates in urine and blood. The variability of biotin responsiveness and the diversity of clinical presentation in the patients originally thought to have a deficiency of beta methylcrotonylCoA carboxylase, a biotin-dependent enzyme, raises the question of a separate, specific apocarboxylase defect.

Amino Acid Metabolism, Inborn Errors↗

Evaluation of continuous solvent extraction of organic acids from biological fluids.

We evaluated the efficiency of continuous solvent extraction with ether for the analysis of organic acids by gas chromatography using a representative group of organic acids and urine from several normal children. Variables examined were the time of extraction, volume of sample, and the quantity and chemical class of acid present. In terms of the decreased time required and invariant extraction of acids of pathologic significnace continuous solvent extraction compares favorably with the more time consuming albeit less discriminatory ion-exchange procedure. Continuous solvent extraction appears especially well suited for analysis of short chain aliphatic acids by gas chromatography.

Body Fluids↗

Glycine therapy in isovaleric acidemia.

The therapeutic efficacy of oral glycine was tested in a 3-year-old girl with isovaleric acidemia. An oral leucine load (25 mg/kg) caused a rise of the blood levels of isovaleric, lactic, and pyruvic acids as well as an increase of urinary excretion of the ketone bodies. These changes did not occur when oral glycine (250 mg/kg) was given with the leucine. Glycine supplementation favored the formation of isovalerylglycine, a nontoxic conjugate of isovaleric acid which is excreted rapidly. Excretion of isovalerylglycine rose threefold when leucine and glycine were administered simultaneously. Chronic glycine therapy was tolerated well and may have prevented one episode of ketoacidosis.

Acidosis↗