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

VernacularTitle:苯丙酮尿症新生儿血斑中辅酶及葡萄糖等物质的分析
Author: Jing YAN ; Chunyan WANG ; Wenyan ZHANG ; Zifeng PI ; Zhiqiang LIU ; Fengrui SONG
Keywords: Phenylketonuria; Coenzyme Q10; Cholesterol; Glucose
From: Chinese Journal of Analytical Chemistry 2017;45(4):574-578
CountryChina
Language:Chinese
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.