Phenylalanine morphological change

Morphological Change Mechanism of a-L-Glutamic Acid with Inclusion of L-Phenylalanine... 

The typical symptoms of untreated individuals with PKU are the manifestation of the neurotoxic effect of phenylalanine on the central nervous system. A morphological change in the brain in patients with PKU affects both white and gray matter. Microcephaly, where the brain mass can be 80 % of that of a healthy individual, is characteristic feature for many untreated PKU patients [37]. This symptom is caused by myelin structure anomalies that result in a loss of myelin volume, disturbances in cortical neuronal development, diffuse cortical atrophy, and general abnormalities in protein synthesis [37, 39, 40]. 

The etiology of brain damage in PKU has not been fully elucidated however, high blood phenylalanine concentrations are associated with changes in brain morphology (gray and white matter) and decreased neurotransmitter synthesis. 

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