Biochemical study of malnutrition. Part V. Metabolism of phenylalanine and tyrosine.
In order to elucidate the metabolic disorders which were observed for phenylalanine and tyrosine in protein-energy malnutrition, loads of both these amino acids were given to young mothers who showed all the clinical and biochemical symptoms of malnutrition and to healthy controls of the same age. Loads of phenylalanine resulted in higher blood levels, lower blood tyrosine formed from phenylalanine and higher urinary excretion of secondary phenylalanine and tyrosine metabolites in the patients, the former being due to a phenylalanine hydroxylase deficiency, the latter to concomitant disturbances in tyrosine catabolism. Loads of tyrosine resulted in very high urinary excretion of secondary tyrosine metabolites in two patients, due to a p-hydroxyphenylalanine-oxidase deficiency and to a high urinary excretion of tyrosine but almost normal excretion of secondary metabolites in the third patient, which is very likely to be due to a tyrosine-transaminase deficiency. Blood tyrosine levels remained lower in all patients, which may be explained by tyrosine malabsorption and increased utilization by the depleted tissues. Protein-energy malnutrition leads to enzyme deficiencies similar to those observed in some inborn errors of metabolism.
D015111:4-Hydroxyphenylpyruvate Dioxygenase / Q000172:deficiency; D000328:Adult; D015023:Democratic Republic of the Congo; D005260:Female; D006801:Humans; D010649:Phenylalanine / Q000097:blood / Q000378:metabolism* / Q000652:urine; D010651:Phenylalanine Hydroxylase / Q000172:deficiency; D011502:Protein-Energy Malnutrition / Q000209:etiology / Q000378:metabolism*; D014443:Tyrosine / Q000097:blood / Q000378:metabolism* / Q000652:urine; D014444:Tyrosine Transaminase / Q000172:deficiency
D014443:Tyrosine; D010649:Phenylalanine; D015111:4-Hydroxyphenylpyruvate Dioxygenase; D010651:Phenylalanine Hydroxylase; D014444:Tyrosine Transaminase