Glutamine in the Phenylketonuric Central Nervous System

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Abstract
Concentrations of glutamine in 31 untreated phenylketonuric adults were decreased in the serum but increased in cerebrospinal fluid (131 per cent of nonphenylketonuric values) and in cerebral white matter (in three patients, 155 per cent of control values). Tyrosine, serine and histidine concentrations in the cerebrospinal fluid were also increased, whereas threonine was significantly lower in untreated phenylketonuria. Of the amino acids reported, only tyrosine was consistently lower in brain tissue.