Article
Pathologic and immunohistochemical findings in hypothalamic and mesencephalic regions in the pah(enu2) mouse model for phenylketonuria.
Pediatric research - 1 Aug 2005
Embury Jennifer E, Reep Roger R, Laipis Philip J
Abstract excerpt
The Pah(enu2) mouse, created through ethylnitrosurea mutagenesis, is a model for phenylketonuria. These mice have elevated serum phenylalanine levels, hypopigmentation, and behavior and movement abnormalities, and female mice exhibit a maternal phenylketonuria syndrome. We evaluated the brains of adult and juvenile Pah(enu2) mice for consistent, demonstrable lesions to elucidate various neuropathologic processes...
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