Article
Phenylketonuria: Direct and indirect effects of phenylalanine.
Experimental neurology - 1 Jul 2016
Schlegel Gudrun, Scholz Ralf, Ullrich Kurt, Santer René, Rune Gabriele M
Abstract excerpt
High phenylalanine concentrations in the brain due to dysfunctional phenylalanine hydroxylase (Pah) are considered to account for mental retardation in phenylketonuria (PKU). In this study, we treated hippocampal cultures with the amino acid in order to determine the role of elevated levels of phenylalanine in PKU-related mental retardation. Synapse density and dendritic length were dramatically reduced in...
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