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PKU is an inherited metabolic disease elicited by the deficiency of phenylalanine hydroxylase. Accumulation of\n                    <jats:sc>l<\/jats:sc>\n                    \u2010phenylalanine (Phe) and its metabolites is found in tissues and body fluids in phenylketonuric patients. In order to mitigate the clinical findings, rigorous dietary Phe restriction constitutes the core of therapeutic management in PKU. Myelination is the process whereby the oligodendrocytes wrap myelin sheaths around the axons, supporting the conduction of action potentials. White matter injuries are implicated in the brain damage related to PKU, especially in untreated or poorly treated patients. The present review summarizes evidence toward putative mechanisms driving the white matter pathology in PKU patients.\n                  <\/jats:p>","DOI":"10.1002\/jnr.24598","type":"journal-article","created":{"date-parts":[[2020,3,6]],"date-time":"2020-03-06T04:04:51Z","timestamp":1583467491000},"page":"349-360","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["White matter disturbances in phenylketonuria: Possible underlying mechanisms"],"prefix":"10.1002","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3329-9972","authenticated-orcid":false,"given":"Bruna Klippel","family":"Ferreira","sequence":"first","affiliation":[{"name":"Laborat\u00f3rio de Neuroenerg\u00e9tica e Erros Inatos do Metabolismo Programa de Bioqu\u00edmica e Biof\u00edsica Celular Instituto de Bioqu\u00edmica M\u00e9dica Leopoldo de Meis Universidade Federal do Rio de Janeiro Porto Alegre 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