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Plasma Phospholipidomic Profile Differs between Children with Phenylketonuria and Healthy Children.
Guerra, Inês M S; Diogo, Luísa; Pinho, Marisa; Melo, Tânia; Domingues, Pedro; Domingues, M Rosário; Moreira, Ana S P.
Afiliación
  • Guerra IMS; Mass Spectrometry Center, LAQV-REQUIMTE, Department of Chemistry, University of Aveiro, Campus Universitário de Santiago, 3810-193 Aveiro, Portugal.
  • Diogo L; Reference Center of Inherited Metabolic Diseases, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, 3000-075 Coimbra, Portugal.
  • Pinho M; ECOMARE, CESAM-Center for Environmental and Marine Studies, Department of Biology, University of Aveiro, Campus de Santiago, 3810-193 Aveiro, Portugal.
  • Melo T; Mass Spectrometry Center, LAQV-REQUIMTE, Department of Chemistry, University of Aveiro, Campus Universitário de Santiago, 3810-193 Aveiro, Portugal.
  • Domingues P; CESAM-Centre for Environmental and Marine Studies, Department of Chemistry, University of Aveiro, Campus Universitário de Santiago, 3810-193 Aveiro, Portugal.
  • Domingues MR; Mass Spectrometry Center, LAQV-REQUIMTE, Department of Chemistry, University of Aveiro, Campus Universitário de Santiago, 3810-193 Aveiro, Portugal.
  • Moreira ASP; Mass Spectrometry Center, LAQV-REQUIMTE, Department of Chemistry, University of Aveiro, Campus Universitário de Santiago, 3810-193 Aveiro, Portugal.
J Proteome Res ; 20(5): 2651-2661, 2021 05 07.
Article en En | MEDLINE | ID: mdl-33819046
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fenilcetonurias Tipo de estudio: Diagnostic_studies / Prognostic_studies Límite: Child / Humans Idioma: En Revista: J Proteome Res Asunto de la revista: BIOQUIMICA Año: 2021 Tipo del documento: Article País de afiliación: Portugal Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fenilcetonurias Tipo de estudio: Diagnostic_studies / Prognostic_studies Límite: Child / Humans Idioma: En Revista: J Proteome Res Asunto de la revista: BIOQUIMICA Año: 2021 Tipo del documento: Article País de afiliación: Portugal Pais de publicación: Estados Unidos