Inhibition byl‐phenylalanine of tryptophan transport by synaptosomal plasma membrane vesicles: Implications in the pathogenesis of phenylketonuria

E Herrero, MC Aragon, C Gimenez… - Journal of Inherited …, 1983 - Wiley Online Library
E Herrero, MC Aragon, C Gimenez, F Valdivieso
Journal of Inherited Metabolic Disease, 1983Wiley Online Library
Phenylalanine is accumulated in the genetically linked deficiency phenylketonuria. The
effect ofl‐phenylalanine on the transport of tryptophan was studied using membrane
vesicles from rat‐brain synaptosomes. Phenylalanine at similar concentrations to those
found in phenylketonuric patients competitively inhibits tryptophan uptake, with aKi of the
same order as theKm for tryptophan. This inhibition could be responsible for the depletion of
serotonin found in phenylketonuria.
Abstract
Phenylalanine is accumulated in the genetically linked deficiency phenylketonuria. The effect ofl‐phenylalanine on the transport of tryptophan was studied using membrane vesicles from rat‐brain synaptosomes. Phenylalanine at similar concentrations to those found in phenylketonuric patients competitively inhibits tryptophan uptake, with aKi of the same order as theKm for tryptophan. This inhibition could be responsible for the depletion of serotonin found in phenylketonuria.
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