苯丙酮尿症新生儿血斑中辅酶及葡萄糖等物质的分析

引用本文: 闫静, 王春艳, 张雯艳, 皮子凤, 刘志强, 宋凤瑞. 苯丙酮尿症新生儿血斑中辅酶及葡萄糖等物质的分析[J]. 分析化学, 2017, 45(4): 574-578. doi: 10.11895/j.issn.0253-3820.160707 shu

Citation: YAN Jing, WANG Chun-Yan, ZHANG Wen-Yan, PI Zi-Feng, LIU Zhi-Qiang, SONG Feng-Rui. Analysis of Coenzyme Q10 and Glucose in Dried Blood Spot from Phenylketonuria Newborn[J]. Chinese Journal of Analytical Chemistry, 2017, 45(4): 574-578. doi: 10.11895/j.issn.0253-3820.160707 shu

苯丙酮尿症新生儿血斑中辅酶及葡萄糖等物质的分析

闫静 ^2, ,
王春艳 ^1, ,
张雯艳 ^3, ,
皮子凤 ^1, ,
刘志强 ^1, ,
宋凤瑞 ^1,

1.
中国科学院长春应用化学研究所长春质谱中心, 长春 130022;
2.
黑龙江中医药大学, 哈尔滨 150040;
3.
吉林省妇幼保健院, 吉林省新生儿筛查中心, 长春 130061
基金项目:
本文系国家自然科学基金资助项目（No.21673219）

摘要: 苯丙酮尿症（PKU）是新生儿先天性苯丙氨酸羟化酶缺陷所引起的苯丙氨酸代谢障碍疾病。本研究采用超高效液相色谱-质谱联用技术，测定了5例PKU新生儿出生3天和出生30天后的血斑与20例年龄相仿的正常新生儿血斑中辅酶Q10的绝对含量和辅酶Q9的相对含量，其中，健康新生儿血斑中辅酶Q10的含量为（122.1±24.9） ng/mL，PKU新生儿组的含量为（59.0±12.0） ng/mL。采用气相色谱-质谱联用技术测定了胆固醇和葡萄糖的相对含量。研究结果表明，与对照组相比，PKU新生儿血斑中辅酶Q10、Q9、胆固醇和葡萄糖的含量均显著降低，辅酶Q10的降低与血斑中苯丙氨酸含量升高呈现显著反向相关。本研究结果为PKU患儿的饮食治疗方案提供了依据。

关键词:

English

Analysis of Coenzyme Q10 and Glucose in Dried Blood Spot from Phenylketonuria Newborn

1.
National Center of Mass Spectrometry in Changchun, Changchun Institute of Applied Chemistry, Chinese Academy of Sciences, Changchun 130022, China;
2.
Heilongjiang University of Chinese Medicine, Haerbin 150040, China;
3.
Jilin Center of Newborn Screening, Jilin Maternal and Child Health Hospital, Changchun 130061, China
Received Date: 20 September 2016
Revised Date: 26 December 2016
Fund Project:
This work was supported by the National Natural Science Foundation of China (No.21673219).

Abstract: Phenylketonuria (PKU) is a newborn inherited metabolic disorder caused by the genetic deficiency of hepatic enzyme phenylalanine hydroxylase (PAH) which thus in metabolic disorder of phenylalanine. In this study, ultra performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) method was used to analyze the accurate amount of coenzyme Q10 (CoQ10) and the relative amount of CoQ9 in newborn dried blood spot (DBS) collected from 5 PKU newborns (3 and 30 days after birth, respectively) and 20 healthy newborns. The content of CoQ10 was (122.1±24.9 ng/mL) and (59.0±12.0 ng/mL) in DBS from healthy newborns and PKU newborns, respectively. The relative contents of cholesterol and glucose in the DBS were determined by gas chromatography-mass spectrometry (GC-MS). In comparison with healthy newborn group, the levels of CoQ10, CoQ9, cholesterol and glucose were all significantly decreased in PKU newborns. The increased content of Phe and the decreased content of CoQ10 showed significant inverse correlation in the DBS from PKU. This study provides references for diet therapy of PKU newborns.

Key words:

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苯丙酮尿症新生儿血斑中辅酶及葡萄糖等物质的分析

English

Analysis of Coenzyme Q10 and Glucose in Dried Blood Spot from Phenylketonuria Newborn

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中国化学会秘书处

苯丙酮尿症新生儿血斑中辅酶及葡萄糖等物质的分析

English

Analysis of Coenzyme Q10 and Glucose in Dried Blood Spot from Phenylketonuria Newborn

CCS Chemistry：嵌段共聚物自组装成 “三明治”二维有机材料，实现纳米级压力传感功能

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CCS Chemistry：工作高效不疲劳，只须让催化剂动起来

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CCS Chemistry：金黄色葡萄球菌醛基脱氢酶是如何识别特异性底物的？

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