Abstract #3592

Diffusion Tensor Imaging of Phenylketonuria (PKU) Mouse Brain White Matter Integrity

Xia Ge¹, Zoe W Hawks², Desiree A White², Auro Ferreiro³, Gautam Dantas³, John Engelbach¹, Joshua S Shimony¹, and Joel R Garbow^1,4

¹Mallinckrodt Institute of Radiology, Washington University, St Louis, MO, United States, ²Department of Psychological and Brain Sciences, Washington University, St Louis, MO, United States, ³The Edison Family Center for Genome Sciences & Systems Biology and Department of Biomedical Engineering, Washington University, St Louis, MO, United States, ⁴Alvin J Siteman Cancer Center, Washington University, St Louis, MO, United States

DTI was used to evaluate brain white-matter integrity and development in Pahenu2 mice, a model of phenylketonuria (PKU). At two months of age, the volume of the CC in Pahenu2 mice was smaller than that of C57Bl6 controls, and the FA of white matter is significantly lower. For PKU mice on a low-Phe diet, both Daxial and FA of CC and IC increased from two to four months, while these metrics decreased in Pahenu2 mice on a normal diet, suggesting a loss of axonal integrity. Our data suggest that Phe levels are critical to white-matter development in Pahenu2 mice.

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