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Regionally selective decreases in cerebral glucose metabolism in a mouse model of phenylketonuria

  • Original Article
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Journal of Inherited Metabolic Disease

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.

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Abbreviations

DG:

deoxyglucose

HMZ:

homozygous

HTZ:

heterozygous

i.v.:

intravenous

PKU:

phenylketonuria

rCMRglc :

regional cerebral metabolic rate for glucose

WT:

wild-type

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Authors and Affiliations

  1. Laboratory of Cerebral Metabolism, Department of Health and Human Services, National Institute of Health, United States Public Health Service, Bethesda, MD, USA

    M. Qin & C. Beebe Smith

  2. Unit on Neuroadaptation and Protein Metabolism, Laboratory of Cerebral Metabolism, National Institute of Health, Bldg. 10, Rm. 2D56 10 Center Drive, Bethesda, MD, 20892-1298, USA

    C. Beebe Smith

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  1. M. Qin
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  2. C. Beebe Smith
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Correspondence to C. Beebe Smith.

Additional information

Communicating editor: Michael Gibson

Competing interests: None declared

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Qin, M., Beebe Smith, C. Regionally selective decreases in cerebral glucose metabolism in a mouse model of phenylketonuria. J Inherit Metab Dis 30, 318–325 (2007). https://doi.org/10.1007/s10545-007-0583-1

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  • DOI: https://doi.org/10.1007/s10545-007-0583-1

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