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L-苯丙氨酸对突触体细胞膜囊泡转运色氨酸的抑制作用:对苯丙酮尿症发病机制的影响

Inhibition by L-phenylalanine of tryptophan transport by synaptosomal plasma membrane vesicles: implications in the pathogenesis of phenylketonuria.

作者信息

Herrero E, Aragon M C, Gimenez C, Valdivieso F

出版信息

J Inherit Metab Dis. 1983;6(1):32-5. doi: 10.1007/BF02391190.

Abstract

Phenylalanine is accumulated in the genetically linked deficiency phenylketonuria. The effect of L-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 a Ki of the same order as the Km for tryptophan. This inhibition could be responsible for the depletion of serotonin found in phenylketonuria.

摘要

苯丙氨酸在与基因相关的苯丙酮尿症中会蓄积。利用大鼠脑突触体的膜囊泡研究了L-苯丙氨酸对色氨酸转运的影响。与苯丙酮尿症患者体内浓度相似的苯丙氨酸竞争性抑制色氨酸摄取,其抑制常数(Ki)与色氨酸的米氏常数(Km)处于同一数量级。这种抑制作用可能是导致苯丙酮尿症中血清素缺乏的原因。

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