高苯丙氨酸血症对苯丙酮尿症患者体内钠钾ATP酶、镁ATP酶活性及生物胺浓度的影响

In vivo effects of high phenylalanine blood levels on Na+,K+-ATPase, Mg2+-ATPase activities and biogenic amine concentrations in phenylketonuria.

作者信息

Schulpis Kleopatra H, Tjamouranis Joanna, Karikas George A, Michelakakis Helen, Tsakiris Stylianos

机构信息

Institute of Child Health, "Aghia Sophia" Children's Hospital, Athens, Greece.

出版信息

Clin Biochem. 2002 Jun;35(4):281-5. doi: 10.1016/s0009-9120(02)00311-9.

DOI:10.1016/s0009-9120(02)00311-9

PMID:12135689

Abstract

OBJECTIVE

To evaluate the activities of Na+,K+-ATPase and Mg2+-ATPase in erythrocyte membranes from phenylketonuric (PKU) patients and to correlate the enzyme activities with their blood phenylalanine (Phe) levels, biogenic amines as well as with their precursors tyrosine (Tyr) and tryptophan (Try).

DESIGN AND METHODS

Twenty three PKU patients were divided into group A (n = 12) on a restricted diet (Phe 1.57 +/- 0.52 mg/dL or 0.10 +/- 0.03 mM) and group B (n = 11) on a "loose" diet (Phe 24.45 +/- 1.50 mg/dL or 1.72 +/- 0.09 mM). The enzyme activities were measured spectrophotometrically, the amino acids with an automatic amino analyser and the biogenic amines with HPLC methods.

RESULTS

In group B, plasma amino acids (Tyr, Try), their biogenic amines [adrenaline (A), noradrenaline (NA), dopamine (DA) and serotonin (5HT)], (Na+,K+)-ATPase and Mg2+-ATPase activities were found remarkably decreased (p < 0.001).

CONCLUSIONS

High Phe and/or low NA, DA, 5HT plasma levels may indirectly inhibit the erythrocyte membrane Na+,K+-ATPase and Mg2+-ATPase in PKU patients. The observed enzyme inhibitions could be a very informative peripheral marker as regards the neurotoxic Phe brain effects.

摘要

目的

评估苯丙酮尿症（PKU）患者红细胞膜中Na +，K + -ATP酶和Mg2 + -ATP酶的活性，并将酶活性与其血苯丙氨酸（Phe）水平、生物胺及其前体酪氨酸（Tyr）和色氨酸（Try）相关联。

设计与方法

23例PKU患者被分为A组（n = 12），采用严格饮食（Phe 1.57±0.52mg/dL或0.10±0.03mM），B组（n = 11）采用“宽松”饮食（Phe 24.45±1.50mg/dL或1.72±0.09mM）。用分光光度法测量酶活性，用自动氨基酸分析仪测量氨基酸，用HPLC方法测量生物胺。