Literature DB >> 17457692

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

M Qin1, C Beebe Smith.   

Abstract

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.

Entities:  

Mesh：

Substances：

Year:  2007        PMID： 17457692     DOI： 10.1007/s10545-007-0583-1

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  24 in total

1.  Estimation of the number of "true" null hypotheses in multivariate analysis of neuroimaging data.

Authors:  F E Turkheimer; C B Smith; K Schmidt
Journal:  Neuroimage       Date:  2001-05       Impact factor: 6.556

Review 2.  Cellular basis of working memory.

Authors:  P S Goldman-Rakic
Journal:  Neuron       Date:  1995-03       Impact factor: 17.173

3.  Cognitive deficits in a genetic mouse model of the most common biochemical cause of human mental retardation.

Authors:  L Zagreda; J Goodman; D P Druin; D McDonald; A Diamond
Journal:  J Neurosci       Date:  1999-07-15       Impact factor: 6.167

4.  Characterization of mutations at the mouse phenylalanine hydroxylase locus.

Authors:  J D McDonald; C K Charlton
Journal:  Genomics       Date:  1997-02-01       Impact factor: 5.736

5.  Local cerebral glucose utilization in the free moving mouse: a comparison during two stages of the activity-rest cycle.

Authors:  T M Jay; M Jouvet; M H des Rosiers
Journal:  Brain Res       Date:  1985-09-09       Impact factor: 3.252

6.  Coactivation of prefrontal cortex and inferior parietal cortex in working memory tasks revealed by 2DG functional mapping in the rhesus monkey.

Authors:  H R Friedman; P S Goldman-Rakic
Journal:  J Neurosci       Date:  1994-05       Impact factor: 6.167

7.  The behavioral profile of severe mental retardation in a genetic mouse model of phenylketonuria.

Authors:  Simona Cabib; Tiziana Pascucci; Rossella Ventura; Valentino Romano; Stefano Puglisi-Allegra
Journal:  Behav Genet       Date:  2003-05       Impact factor: 2.805

8.  Amygdalectomy and ventromedial prefrontal ablation produce similar deficits in food choice and in simple object discrimination learning for an unseen reward.

Authors:  L L Baylis; D Gaffan
Journal:  Exp Brain Res       Date:  1991       Impact factor: 1.972

9.  Functional specialization within medial frontal cortex of the anterior cingulate for evaluating effort-related decisions.

Authors:  Mark E Walton; David M Bannerman; Karin Alterescu; Matthew F S Rushworth
Journal:  J Neurosci       Date:  2003-07-23       Impact factor: 6.167

10.  Mouse models of human phenylketonuria.

Authors:  A Shedlovsky; J D McDonald; D Symula; W F Dove
Journal:  Genetics       Date:  1993-08       Impact factor: 4.562
View more
  8 in total

1.  A novel nitroxide is an effective brain redox imaging contrast agent and in vivo radioprotector.

Authors:  Ryan M Davis; Anastasia L Sowers; William DeGraff; Marcelino Bernardo; Angela Thetford; Murali C Krishna; James B Mitchell
Journal:  Free Radic Biol Med       Date:  2011-05-25       Impact factor: 7.376

2.  Biochemical, Metabolic, and Behavioral Characteristics of Immature Chronic Hyperphenylalanemic Rats.

Authors:  Gerald A Dienel; Nancy F Cruz
Journal:  Neurochem Res       Date:  2015-07-30       Impact factor: 3.996

3.  Voluntary Exercise Prevents Oxidative Stress in the Brain of Phenylketonuria Mice.

Authors:  Priscila Nicolao Mazzola; Vibeke Bruinenberg; Karen Anjema; Danique van Vliet; Carlos Severo Dutra-Filho; Francjan J van Spronsen; Eddy A van der Zee
Journal:  JIMD Rep       Date:  2015-10-07

4.  Inhibiting neutral amino acid transport for the treatment of phenylketonuria.

Authors:  Adam M Belanger; Malgorzata Przybylska; Estelle Gefteas; Matthew Furgerson; Sarah Geller; Alla Kloss; Seng H Cheng; Yunxiang Zhu; Nelson S Yew
Journal:  JCI Insight       Date:  2018-07-26

Review 5.  Pathogenesis of CNS involvement in disorders of amino and organic acid metabolism.

Authors:  S Kölker; S W Sauer; G F Hoffmann; I Müller; M A Morath; J G Okun
Journal:  J Inherit Metab Dis       Date:  2008-04-04       Impact factor: 4.982

Review 6.  Phenylketonuria.

Authors:  Francjan J van Spronsen; Nenad Blau; Cary Harding; Alberto Burlina; Nicola Longo; Annet M Bosch
Journal:  Nat Rev Dis Primers       Date:  2021-05-20       Impact factor: 52.329

7.  A Retrospective Case Series Analysis of the Relationship Between Phenylalanine: Tyrosine Ratio and Cerebral Glucose Metabolism in Classical Phenylketonuria and Hyperphenylalaninemia.

Authors:  Colm J McGinnity; Daniela A Riaño Barros; Eric Guedj; Nadine Girard; Christopher Symeon; Helen Walker; Sally F Barrington; Mary Summers; Mervi Pitkanen; Yusof Rahman
Journal:  Front Neurosci       Date:  2021-06-17       Impact factor: 4.677

8.  Altered brain protein expression profiles are associated with molecular neurological dysfunction in the PKU mouse model.

Authors:  Esther Imperlini; Stefania Orrù; Claudia Corbo; Aurora Daniele; Francesco Salvatore
Journal:  J Neurochem       Date:  2014-03-24       Impact factor: 5.372
  8 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.