Plasma Phospholipidomic Profile Differs between Children with Phenylketonuria and Healthy Children
authors Guerra, IMS; Diogo, L; Pinho, M; Melo, T; Domingues, P; Domingues, MR; Moreira, ASP
nationality International
journal JOURNAL OF PROTEOME RESEARCH
author keywords inborn errors of metabolism; phenylketonuria; plasma; phospholipids; lipidomics; mass spectrometry
keywords POLYUNSATURATED FATTY-ACIDS; DOCOSAHEXAENOIC ACID; SUPPLEMENTATION; CHOLESTEROL; LIPIDS; N-3; PHENYLALANINE; ADOLESCENTS; INFANTS; ADULTS
abstract Phenylketonuria (PKU) is a disease of the catabolism of phenylalanine (Phe), caused by an impaired function of the enzyme phenylalanine hydroxylase. Therapeutics is based on the restriction of Phe intake, which mostly requires a modification of the diet. Dietary restrictions can lead to imbalances in specific nutrients, including lipids. In the present study, the plasma phospholipidome of PKU and healthy children (CT) was analyzed by hydrophilic interaction liquid chromatography-tandem mass spectrometry and gas chromatography-mass spectrometry. Using this approach, 187 lipid species belonging to nine different phospholipid classes and three ceramides were identified. Principal component analysis of the lipid species data set showed a distinction between PKU and CT groups. Univariate analysis revealed that 146 species of phospholipids were significantly different between both groups. Lipid species showing significant variation included phosphatidylcholines, containing polyunsaturated fatty acids (PUFA), which were more abundant in PKU. The high level of PUFA-containing lipid species in children with PKU may be related to a diet supplemented with PUFA. This study was the first report comparing the plasma polar lipidome of PKU and healthy children, highlighting that the phospholipidome of PKU children is significantly altered compared to CT. However, further studies with larger cohorts are needed to clarify whether these changes are specific to phenylketonuric children.
publisher AMER CHEMICAL SOC
issn 1535-3893
isbn 1535-3907
year published 2021
volume 20
issue 5
beginning page 2651
ending page 2661
digital object identifier (doi) 10.1021/acs.jproteome.0c01052
web of science category 11
subject category Biochemical Research Methods
unique article identifier WOS:000649269600043
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