High phenylalanine concentrations induce demyelination and microglial activation in mouse cerebellar organotypic slices

Phenylketonuria (PKU) is an inborn error of metabolism. Mutations in the enzyme phenylalanine hydroxylase (PAH)-encoding gene lead to a decreased metabolism of the amino acid phenylalanine (Phe). The deficiency in PAH increases Phe levels in blood and brain. Accumulation of Phe can lead to delayed d...

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Bibliographic Details
Main Authors: Orli Thau-Zuchman, Patrick N. Pallier, Paul J. M. Savelkoul, Almar A. M. Kuipers, J. Martin Verkuyl, Adina T. Michael-Titus
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-09-01
Series:Frontiers in Neuroscience
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fnins.2022.926023/full