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Regionally selective decreases in cerebral glucose metabolism in a mouse model of phenylketonuria
Author(s) -
Qin M.,
Beebe Smith C.
Publication year - 2007
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-007-0583-1
Subject(s) - hippocampus , cerebral cortex , perirhinal cortex , medicine , endocrinology , neuroscience , cingulate cortex , infralimbic cortex , anterior cingulate cortex , biology , central nervous system , cognition , temporal lobe , prefrontal cortex , epilepsy
Summary Impairment of cognitive function is characteristic of untreated phenylketonuria in humans and in the pah enu2 mouse model of phenylketonuria. We measured regional cerebral metabolic rate for glucose in the adult male pah enu2 mouse to determine the effect of PKU on functional activity in brain and to discern what, if any, brain areas are affected. Our results demonstrate selective decreases (17–21%) in regions thought to be involved in executive function. Regions most significantly affected include prelimbic, anterior cingulate, orbital frontal and perirhinal cortex. Sensory and motor areas of cortex and hippocampus were remarkably unaffected.