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苯丙酮尿症:一种同时测定血浆苯丙氨酸和酪氨酸的新方法。

Phenylketonuria: a new method for the simultaneous determination of plasma phenylalanine and tyrosine.

作者信息

Shen R S, Abell C W

出版信息

Science. 1977 Aug 12;197(4304):665-7. doi: 10.1126/science.877580.

DOI:10.1126/science.877580
PMID:877580
Abstract

This quantitative spectrophotometric method is based on the conversion of phenylalanine and tyrosine by phenylalanine ammonia-lyase to trans-cinnamic acid and trans-coumaric acid, respectively. Neither deproteinization nor prior incubation of the sample is required, and the entire procedure can be performed in 20 minutes. The method is sensitive to 1-micromolar concentrations of the two compounds, and only 20 microliters of plasma or serum is required to determine both phenylalanine and tyrosine simultaneously. These amino acids were determined between molar ratios (phenylalanine to tyrosine) of 0.1 to 40 in the serum or plasma of healthy individuals and plasma of phenylketonuric patients.

摘要

这种定量分光光度法基于苯丙氨酸解氨酶将苯丙氨酸和酪氨酸分别转化为反式肉桂酸和反式香豆酸。该方法无需对样品进行脱蛋白处理或预先孵育,整个过程可在20分钟内完成。该方法对两种化合物的1微摩尔浓度敏感,同时测定苯丙氨酸和酪氨酸仅需20微升血浆或血清。在健康个体的血清或血浆以及苯丙酮尿症患者的血浆中,这些氨基酸在摩尔比(苯丙氨酸与酪氨酸)为0.1至40的范围内进行测定。

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Phenylketonuria: a new method for the simultaneous determination of plasma phenylalanine and tyrosine.苯丙酮尿症:一种同时测定血浆苯丙氨酸和酪氨酸的新方法。
Science. 1977 Aug 12;197(4304):665-7. doi: 10.1126/science.877580.
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引用本文的文献

1
Predicting the phenylalanine blood concentration from urine analyses. An approach to noninvasive monitoring of patients with phenylketonuria.
J Inherit Metab Dis. 2005;28(6):855-61. doi: 10.1007/s10545-005-0160-4.
2
Artificial cell-microencapsulated phenylalanine ammonia-lyase.
Appl Biochem Biotechnol. 1984;10:57-9. doi: 10.1007/BF02783735.
3
hph-1: a mouse mutant with hereditary hyperphenylalaninemia induced by ethylnitrosourea mutagenesis.hph-1:一种由乙基亚硝基脲诱变诱导产生遗传性高苯丙氨酸血症的小鼠突变体。
Genetics. 1988 Feb;118(2):299-305. doi: 10.1093/genetics/118.2.299.