Dramatic brain aminergic deficit in a genetic mouse model of phenylketonuria

Research output: Contribution to journalArticle

63 Citations (Scopus)

Abstract

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. (C) 2000 Lippincott Williams and Wilkins.

Original languageEnglish
Pages (from-to)1361-1364
Number of pages4
JournalNeuroReport
Volume11
Issue number6
Publication statusPublished - Apr 27 2000

Fingerprint

Phenylketonurias
Genetic Models
Prefrontal Cortex
Putamen
Nucleus Accumbens
Amygdala
Dopamine
Serotonin
Brain
Hippocampus
Norepinephrine
Gyrus Cinguli
Intellectual Disability
Catecholamines

Keywords

  • Basal ganglia
  • Catecholamines
  • Mutant mice
  • Phenylalanine hydroxylase
  • Phenylketonuria
  • Serotonin
  • Tryptophan
  • Tyrosine

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Dramatic brain aminergic deficit in a genetic mouse model of phenylketonuria. / Puglisi-Allegra, Stefano; Cabib, Simona; Pascucci, Tiziana; Ventura, Rossella; Calì, Francesco; Romano, Valentino.

In: NeuroReport, Vol. 11, No. 6, 27.04.2000, p. 1361-1364.

Research output: Contribution to journalArticle

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AU - Romano, Valentino

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AB - 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. (C) 2000 Lippincott Williams and Wilkins.

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