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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

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).

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.
- Phenylketonuria,
- Coenzyme Q10,
- Cholesterol,
- Glucose
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