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人类胎儿和儿童体内参与苯丙氨酸代谢的酶。

Enzymes involved in phenylalanine metabolism in the human foetus and child.

作者信息

McLean A, Marwick M J, Clayton B E

出版信息

J Clin Pathol. 1973 Sep;26(9):678-83. doi: 10.1136/jcp.26.9.678.

DOI:10.1136/jcp.26.9.678
PMID:4148075
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC477849/
Abstract

The activities of phenylalanine p-hydroxylase, tyrosine: alpha-ketoglutarate and phenylalanine: pyruvate aminotransferases, and aromatic alpha-ketoacid reductase have been measured in liver biopsies obtained from children with a series of disorders other than phenylketonuria and from human foetuses aged 8.5 to 24 weeks. The importance of such a study in relation to the management of infants with hyperphenylalaninaemia and pregnant phenylketonuric women is discussed

摘要

已对从患有一系列除苯丙酮尿症之外的疾病的儿童以及8.5至24周龄的人类胎儿获取的肝脏活检样本中的苯丙氨酸对羟基化酶、酪氨酸：α-酮戊二酸转氨酶、苯丙氨酸：丙酮酸转氨酶和芳香族α-酮酸还原酶的活性进行了测定。讨论了此类研究对于高苯丙氨酸血症婴儿和患有苯丙酮尿症的孕妇的管理的重要性。

相似文献

1
Enzymes involved in phenylalanine metabolism in the human foetus and child.人类胎儿和儿童体内参与苯丙氨酸代谢的酶。
J Clin Pathol. 1973 Sep;26(9):678-83. doi: 10.1136/jcp.26.9.678.
2
Phenylalanine hydroxylase expression in liver of a fetus with phenylketonuria.
J Pediatr. 1988 Sep;113(3):463-8. doi: 10.1016/s0022-3476(88)80629-2.
3
Changes in phenylalanine tolerance while monitoring the dietetic treatment of pregnant women suffering from hyperphenylalaninaemia.在监测高苯丙氨酸血症孕妇的饮食治疗时苯丙氨酸耐受性的变化。
J Inherit Metab Dis. 1988;11(4):427-8. doi: 10.1007/BF01800434.
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Atypical phenylketonuric heterozygote. Deficiency in phenylalanine hydroxylase and transaminase activity.非典型苯丙酮尿症杂合子。苯丙氨酸羟化酶和转氨酶活性缺乏。
J Pediatr. 1966 Mar;68(3):351-60. doi: 10.1016/s0022-3476(66)80237-8.
5
Diagnosis of phenylalanine hydroxylase deficiency (phenylketonuria).
Am J Dis Child. 1982 Feb;136(2):111-4. doi: 10.1001/archpedi.1982.03970380023006.
6
Influence of age on ortho-hydroxyphenylacetic acid excretion in phenylketonuria and its genetic variants.年龄对苯丙酮尿症及其基因变异型中邻羟基苯乙酸排泄的影响。
Pediatr Res. 1974 May;8(5):540-5. doi: 10.1203/00006450-197405000-00002.
7
[Current problems in the diagnosis and treatment of phenylketonuria].
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Glucagon stimulation of phenylalanine metabolism. The effects of acute and chronic glucagon treatment.胰高血糖素对苯丙氨酸代谢的刺激作用。急性和慢性胰高血糖素治疗的效果。
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[Importance of early diagnosis of phenylketonuria in women and control of phenylalanine levels during pregnancy].[女性苯丙酮尿症早期诊断及孕期苯丙氨酸水平控制的重要性]
Nutr Hosp. 2012 Sep-Oct;27(5):1658-61. doi: 10.3305/nh.2012.27.5.5945.
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Studies on the experimental phenylketonuria in rats.
Tohoku J Exp Med. 1975 Oct;117(2):167-78. doi: 10.1620/tjem.117.167.

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J Mol Med (Berl). 2012 Dec;90(12):1497-504. doi: 10.1007/s00109-012-0930-z. Epub 2012 Jun 24.
2
Problems affecting the community. Population screening.影响社区的问题。人群筛查。
J Clin Pathol Suppl (R Coll Pathol). 1974;8:145-9.

本文引用的文献

1
The amino-acid pattern in human foetal and maternal plasma at delivery.分娩时人胎儿和母体血浆中的氨基酸模式。
Biochem J. 1950 Aug;47(2):223-7. doi: 10.1042/bj0470223.
2
Hepatic metabolism of phenylalanine during development.发育过程中苯丙氨酸的肝脏代谢。
J Clin Invest. 1959 Dec;38(12):2189-96. doi: 10.1172/JCI103998.
3
Factors influencing the development of tyrosine-alpha-ketoglutarate transaminase activity in rat liver.影响大鼠肝脏中酪氨酸-α-酮戊二酸转氨酶活性发展的因素。
J Biol Chem. 1959 Mar;234(3):609-12.
4
Phenylalanine hydroxylation cofactor in phenylketonuria.苯丙酮尿症中的苯丙氨酸羟化辅因子。
Science. 1958 Dec 12;128(3337):1506-8. doi: 10.1126/science.128.3337.1506.
5
The chemical estimation of tyrosine and tyramine.酪氨酸和酪胺的化学测定
J Biol Chem. 1952 May;196(1):227-33.
6
Free amino acids of human foetal and adult liver.人类胎儿和成人肝脏中的游离氨基酸。
Nature. 1966 Oct 15;212(5059):292-3. doi: 10.1038/212292a0.
7
Reduction of phenylpyruvic acids to phenyllactic acids in mammalian tissues.哺乳动物组织中苯丙酮酸还原为苯乳酸。
J Biol Chem. 1966 Mar 25;241(6):1345-9.
8
Characteristics of the hepatic phenylalanine-hydroxylating system in newborn rats.新生大鼠肝脏苯丙氨酸羟化系统的特征
J Biol Chem. 1965 Sep;240(9):3617-22.
9
Phenylalaninaemia or classical phenylketonuria (PKU)?苯丙酮尿症还是经典型苯丙酮尿症（PKU）？
Neuropadiatrie. 1970 Apr;1(4):379-82. doi: 10.1055/s-0028-1091826.
10
Reduction of aromatic alpha-keto acids by lactic dehydrogenase isozymes and aromatic alpha-keto acid reductase.乳酸脱氢酶同工酶和芳香族α-酮酸还原酶对芳香族α-酮酸的还原作用。
Ann N Y Acad Sci. 1968 Jun 14;151(1):627-37. doi: 10.1111/j.1749-6632.1968.tb11923.x.