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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.

DOI:10.1007/BF02391190
PMID:6408306
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。

相似文献

1
Inhibition by L-phenylalanine of tryptophan transport by synaptosomal plasma membrane vesicles: implications in the pathogenesis of phenylketonuria.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
J Inherit Metab Dis. 1983;6(1):32-5. doi: 10.1007/BF02391190.
2
Inhibition by L-phenylalanine of tyrosine transport by synaptosomal plasma membrane vesicles: implications in the pathogenesis of phenylketonuria.L-苯丙氨酸对突触体细胞膜囊泡转运酪氨酸的抑制作用：对苯丙酮尿症发病机制的影响
J Neurochem. 1982 Oct;39(4):1185-7. doi: 10.1111/j.1471-4159.1982.tb11513.x.
3
Tryptophan transport into plasma membrane vesicles derived from rat brain synaptosomes.色氨酸转运至源自大鼠脑突触体的质膜囊泡中。
J Neurochem. 1983 Feb;40(2):332-7. doi: 10.1111/j.1471-4159.1983.tb11287.x.
4
[Inhibition of L-tyrosine transport in isolated synaptosomes from rat brain in the presence of L-tryptophan and L-phenylalanine].[在L-色氨酸和L-苯丙氨酸存在的情况下对大鼠脑分离突触体中L-酪氨酸转运的抑制作用]
Rev Esp Fisiol. 1982;38 Suppl:217-20.
5
Tryptophan uptake and hydroxylation in rat forebrain synaptosomes.大鼠前脑突触体中的色氨酸摄取与羟化作用
J Neurochem. 1984 Mar;42(3):677-84. doi: 10.1111/j.1471-4159.1984.tb02736.x.
6
Chronic administration of lithium modulates tryptophan transport by changing the properties of the synaptosomal plasma membrane.长期服用锂盐可通过改变突触体细胞膜的特性来调节色氨酸转运。
Life Sci. 1987 Aug 3;41(5):643-50. doi: 10.1016/0024-3205(87)90419-x.
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Inhibition of protein and aminoacyl-tRNA synthesis, and binding and transport sites for aromatic amino acids in the brain in vitro with aromatic acids.体外使用芳香酸对大脑中蛋白质和氨酰 - tRNA合成以及芳香族氨基酸的结合和转运位点的抑制作用。
Int J Neurosci. 1984 Mar;23(1):1-13. doi: 10.3109/00207458408985341.
8
Ontogenetic studies on tryptophan transport into plasma membrane vesicles derived from rat brain synaptosomes: effect of thyroid hormones.色氨酸转运至源自大鼠脑突触体的质膜囊泡的个体发生学研究：甲状腺激素的作用。
Neurochem Res. 1985 May;10(5):579-89. doi: 10.1007/BF00964397.
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Tryptophan transport across the synaptosomal membrane.色氨酸跨突触体膜的转运。
J Neurochem. 1970 Sep;17(9):1339-53. doi: 10.1111/j.1471-4159.1970.tb06869.x.
10
Studies on the mechanism by which tryptophan efflux from isolated synaptosomes is stimulated by depolarization.关于去极化刺激分离突触体中色氨酸外流机制的研究。
Br J Pharmacol. 1988 Feb;93(2):341-8. doi: 10.1111/j.1476-5381.1988.tb11440.x.

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The protective effect of L-cysteine and glutathione on the adult and aged rat brain (Na+,K+)-ATPase and Mg2+-ATPase activities in galactosemia in vitro.L-半胱氨酸和谷胱甘肽对体外半乳糖血症成年及老年大鼠脑（Na +，K +）-ATP酶和Mg2 + -ATP酶活性的保护作用。
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本文引用的文献

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Phenylalanine as inhibitor of transport of amino-acids in brain.苯丙氨酸作为大脑中氨基酸转运的抑制剂。
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Studies on aromatic amino acid uptake by rat brain in vivo. Uptake of phenylalanine and of tryptophan; inhibition and stereoselectivity in the uptake of tyrosine by brain and muscle.大鼠脑内芳香族氨基酸摄取的体内研究。苯丙氨酸和色氨酸的摄取；脑和肌肉中酪氨酸摄取的抑制作用及立体选择性。
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半乳糖血症体外模型中乳鼠脑区Na⁺,K⁺-ATP酶活性的分布：L-半胱氨酸和谷胱甘肽的作用
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Pattern reversal visual evoked potentials in phenylketonuria.苯丙酮尿症中的图形翻转视觉诱发电位
J Inherit Metab Dis. 1988;11(4):416-21. doi: 10.1007/BF01800430.
8
Serotonin and noradrenaline concentrations and serotonin uptake in platelets from hyperphenylalaninaemic patients.高苯丙氨酸血症患者血小板中血清素和去甲肾上腺素浓度以及血清素摄取情况
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Eur J Pediatr. 1988 Dec;148(3):238-45. doi: 10.1007/BF00441411.
10
The depletion of tryptophan and serotonin in the brain of developing hyperphenylalaninemic rats is abolished by the additional administration of lysine.在发育中的高苯丙氨酸血症大鼠大脑中，色氨酸和血清素的消耗可通过额外给予赖氨酸而消除。
Neurochem Res. 1986 Dec;11(12):1663-8. doi: 10.1007/BF00967744.
从大鼠脑部分离出的膜囊泡对L-脯氨酸的主动转运
Biochim Biophys Acta. 1980 Jul 16;600(1):185-94. doi: 10.1016/0005-2736(80)90423-x.
4
Tryptophan and phenylalanine transport in rat cerebral cortex slices as influenced by sodium ions.钠离子对大鼠大脑皮层切片中色氨酸和苯丙氨酸转运的影响
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L-Aspartate transport into plasma membrane vesicles derived from rat brain synaptosomes.L-天冬氨酸转运至源自大鼠脑突触体的质膜囊泡中。
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Tyrosine transport by membrane vesicles isolated from rat brain.从大鼠脑部分离出的膜囊泡对酪氨酸的转运
Biochim Biophys Acta. 1981 Sep 7;646(3):465-70. doi: 10.1016/0005-2736(81)90316-3.
8
Inhibition by L-phenylalanine of tyrosine transport by synaptosomal plasma membrane vesicles: implications in the pathogenesis of phenylketonuria.L-苯丙氨酸对突触体细胞膜囊泡转运酪氨酸的抑制作用：对苯丙酮尿症发病机制的影响
J Neurochem. 1982 Oct;39(4):1185-7. doi: 10.1111/j.1471-4159.1982.tb11513.x.
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The influence of high phenylalanine and tyrosine on the concentrations of essential amino acids in brain.高苯丙氨酸和酪氨酸对脑中必需氨基酸浓度的影响。
J Neurochem. 1968 Mar;15(3):235-41. doi: 10.1111/j.1471-4159.1968.tb06202.x.