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Publication : Dramatic brain aminergic deficit in a genetic mouse model of phenylketonuria.

First Author  Puglisi-Allegra S Year  2000
Journal  Neuroreport Volume  11
Issue  6 Pages  1361-4
PubMed ID  10817622 Mgi Jnum  J:103668
Mgi Id  MGI:3610597 Doi  10.1097/00001756-200004270-00042
Citation  Puglisi-Allegra S, et al. (2000) Dramatic brain aminergic deficit in a genetic mouse model of phenylketonuria. Neuroreport 11(6):1361-4
abstractText  Clinical data suggest that brain catecholamines and serotonin are deficient in phenylketonuria (PKU), an inherited metabolic disorder that causes severe mental retardation and neurological disturbances. To test this hypothesis, brain tissue levels of dopamine (DA), norepinephrine (NE), 5-hydroxytryptamine (5-HT) and their metabolites were evaluated in the genetic mouse model of PKU (Pah(enu2)). Results indicated a significant reduction of 5-HT levels and metabolism in prefrontal cortex (pFC), cingulate cortex (Cg), nucleus accumbens (NAc), caudate putamen (CP), hippocampus (HIP) and amygdala (AMY). NE content and metabolism were reduced in pFC, Cg, AMY and HIP. Finally, significantly reduced DA content and metabolism was observed in pFC, NAc, CP and AMY. In pFC, NAc and CP there was also a marked reduction of DA release.
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