Brain White Matter Integrity Mediates the Relationship Between Phenylalanine Control and Executive Abilities in Children with Phenylketonuria
- 22 July 2016
- book chapter
- Published by Springer Nature
- Vol. 33, 41-47
Abstract
We tested the hypothesis that brain white matter integrity mediates the relationship between phenylalanine (Phe) control and executive abilities in children with phenylketonuria (PKU; N = 36). To do so, we examined mean diffusivity (MD) from diffusion tensor imaging (DTI) in two white matter brain regions (posterior parietal–occipital, PPO; centrum semiovale, CSO) and lifetime phenylalanine (Phe) exposure; the executive abilities examined included verbal strategic processing, nonverbal strategic processing, and working memory. Mediation modeling showed that MD in the PPO and CSO mediated the relationship between Phe exposure and nonverbal strategic processing, MD in the CSO mediated the relationship between Phe exposure and verbal strategic processing, and MD in the PPO mediated the relationship between Phe exposure and working memory. These exploratory findings demonstrate the importance of using sophisticated modeling procedures to understand the interplay among metabolic control, neural factors, and functional outcomes in individuals with PKU.Keywords
This publication has 31 references indexed in Scilit:
- White matter integrity and executive abilities in individuals with phenylketonuriaMolecular Genetics and Metabolism, 2013
- Apparent diffusion coefficient restriction in the white matter: going beyond acute brain territorial ischemiaInsights into Imaging, 2011
- Phenylalanine hydroxylase deficiencyGenetics in Medicine, 2011
- Mediation Analysis in Social Psychology: Current Practices and New RecommendationsSocial and Personality Psychology Compass, 2011
- Correlation of age‐specific phenylalanine levels with intellectual outcome in patients with phenylketonuriaJournal of Inherited Metabolic Disease, 2011
- Pathogenesis of cognitive dysfunction in phenylketonuria: Review of hypotheses☆Molecular Genetics and Metabolism, 2010
- Age-related decline in the microstructural integrity of white matter in children with early- and continuously-treated PKU: A DTI study of the corpus callosum☆Molecular Genetics and Metabolism, 2010
- Human brain white matter atlas: Identification and assignment of common anatomical structures in superficial white matterNeuroImage, 2008
- Are Neuropsychological Impairments in Children with Early-Treated Phenylketonuria (PKU) Related to White Matter Abnormalities or Elevated Phenylalanine Levels?Developmental Neuropsychology, 2007
- ThePAH gene, phenylketonuria, and a paradigm shiftHuman Mutation, 2007