Published in Mol Genet Metab on January 01, 2010
5-Hydroxytryptophan during critical postnatal period improves cognitive performances and promotes dendritic spine maturation in genetic mouse model of phenylketonuria. Int J Neuropsychopharmacol (2010) 0.86
Large Neutral Amino Acid Supplementation Exerts Its Effect through Three Synergistic Mechanisms: Proof of Principle in Phenylketonuria Mice. PLoS One (2015) 0.86
Differential effects of low-phenylalanine protein sources on brain neurotransmitters and behavior in C57Bl/6-Pah(enu2) mice. Mol Genet Metab (2014) 0.85
Altered brain protein expression profiles are associated with molecular neurological dysfunction in the PKU mouse model. J Neurochem (2014) 0.84
In vivo catecholaminergic metabolism in the medial prefrontal cortex of ENU2 mice: an investigation of the cortical dopamine deficit in phenylketonuria. J Inherit Metab Dis (2012) 0.81
Behavioral and neurochemical characterization of new mouse model of hyperphenylalaninemia. PLoS One (2013) 0.80
Processing speed and executive abilities in children with phenylketonuria. Neuropsychology (2012) 0.78
Animal models of human genetic diseases: do they need to be faithful to be useful? Mol Genet Genomics (2011) 0.77
Biochemical, Metabolic, and Behavioral Characteristics of Immature Chronic Hyperphenylalanemic Rats. Neurochem Res (2015) 0.75
A Multiplatform Metabolomics Approach to Characterize Plasma Levels of Phenylalanine and Tyrosine in Phenylketonuria. JIMD Rep (2016) 0.75
Early-onset behavioral and neurochemical deficits in the genetic mouse model of phenylketonuria. PLoS One (2017) 0.75
Associative learning elicits the formation of multiple-synapse boutons. J Neurosci (2001) 1.68
Prevalence of congenital heart disease in patients with phenylketonuria. J Pediatr (1991) 1.44
Pathogenesis of cognitive dysfunction in phenylketonuria: review of hypotheses. Mol Genet Metab (2010) 1.38
Impaired prenatal and postnatal growth in Dutch patients with phenylketonuria. The National PKU Steering Committee. Arch Dis Child (1994) 1.14
The cholinergic system, circadian rhythmicity, and time memory. Behav Brain Res (2010) 1.10
The course of life and quality of life of early and continuously treated Dutch patients with phenylketonuria. J Inherit Metab Dis (2006) 1.08
Sustained attention and inhibition of cognitive interference in treated phenylketonuria: associations with concurrent and lifetime phenylalanine concentrations. Neuropsychologia (2002) 1.07
Clinical and diagnostic approach in unsolved CDG patients with a type 2 transferrin pattern. Biochim Biophys Acta (2011) 1.03
Adherence issues in inherited metabolic disorders treated by low natural protein diets. Ann Nutr Metab (2012) 0.99
Behaviour and school achievement in patients with early and continuously treated phenylketonuria. J Inherit Metab Dis (2000) 0.98
Increased amyloid precursor protein expression and serotonergic sprouting following excitotoxic lesion of the rat magnocellular nucleus basalis: neuroprotection by Ca(2+) antagonist nimodipine. Neuroscience (2000) 0.97
Behavioural factors related to metabolic control in patients with phenylketonuria. J Inherit Metab Dis (2005) 0.93
The neuropsychological profile of early and continuously treated phenylketonuria: orienting, vigilance, and maintenance versus manipulation-functions of working memory. Neurosci Biobehav Rev (2002) 0.93
Renal function in tyrosinaemia type I after liver transplantation: a long-term follow-up. J Inherit Metab Dis (2005) 0.92
Dynamics in the ultrastructure of asymmetric axospinous synapses in the frontal cortex of hibernating European ground squirrels (Spermophilus citellus). Synapse (2007) 0.90
Gradual reappearance of post-hibernation circadian rhythmicity correlates with numbers of vasopressin-containing neurons in the suprachiasmatic nuclei of European ground squirrels. J Comp Physiol B (2002) 0.89
Normal very-long-chain fatty acids in peroxisomal D-bifunctional protein deficiency: a diagnostic pitfall. J Inherit Metab Dis (2005) 0.88
Chronic but not acute foot-shock stress leads to temporary suppression of cell proliferation in rat hippocampus. Neuroscience (2009) 0.88
Tyrosinemia type I treated by NTBC: how does AFP predict liver cancer? Mol Genet Metab (2006) 0.87
Information processing in patients with early and continuously-treated phenylketonuria. Eur J Pediatr (1995) 0.87
Age-related loss of calcium binding proteins in rabbit hippocampus. Neurobiol Aging (1996) 0.85
MR spectroscopy of the brain in Leigh syndrome. Brain Dev (2008) 0.83
Plasma phenylalanine in patients with phenylketonuria self-managing their diet. Arch Dis Child (2005) 0.82
17beta-estradiol enhances cortical cholinergic innervation and preserves synaptic density following excitotoxic lesions to the rat nucleus basalis magnocellularis. Neuroscience (2002) 0.82
1H MR spectroscopy of the brain in Cr transporter defect. Mol Genet Metab (2005) 0.82
A patient with lethal cardiomyopathy and a carnitine-acylcarnitine translocase deficiency. J Inherit Metab Dis (1995) 0.82
Predictive validity of a non-induced mouse model of compulsive-like behavior. Behav Brain Res (2011) 0.82
Expression of circadian rhythmicity correlates with the number of arginine-vasopressin-immunoreactive cells in the suprachiasmatic nucleus of common voles, Microtus arvalis. Brain Res (1994) 0.82
Short-term dietary interventions in children and adolescents with treated phenylketonuria: effects on neuropsychological outcome of a well-controlled population. J Inherit Metab Dis (2002) 0.81
The 48-hour tetrahydrobiopterin loading test in patients with phenylketonuria: evaluation of protocol and influence of baseline phenylalanine concentration. Mol Genet Metab (2011) 0.81
Co-expression of Fos immunoreactivity in protein kinase (PKC gamma)-positive neurones: quantitative analysis of a brain region involved in learning. Brain Res (1993) 0.81
Circadian rhythms differ between selected mouse lines: a model to study the role of vasopressin neurons in the suprachiasmatic nuclei. Brain Res Bull (1993) 0.80
Liver transplantation in tyrosinaemia type I: the Groningen experience. J Inherit Metab Dis (1995) 0.80
Ultrastructural localization of cholinergic muscarinic receptors in rat brain cortical capillaries. Brain Res (1996) 0.79
Circadian clocks and memory: time-place learning. Front Mol Neurosci (2013) 0.79
Circadian dynamics of vasopressin in mouse selection lines: translation and release in the SCN. Brain Res (2005) 0.79
The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria. J Inherit Metab Dis (2005) 0.79
Neuroprotection against NMDA induced cell death in rat nucleus basalis by Ca2+ antagonist nimodipine, influence of aging and developmental drug treatment. Neurodegeneration (1995) 0.78
The psychology and neuropathology of phenylketonuria. Mol Genet Metab (2010) 0.78
Recurrent thrombo-embolism in a child with a congenital disorder of glycosylation (CDG) type Ib and treatment with mannose. Pediatr Hematol Oncol (2008) 0.78
Being circadian or not: vasopressin release in cultured SCN mirrors behavior in adult voles. Neuroreport (2000) 0.78
Non-selectivity of the monoclonal antibody M35 for subtypes of muscarinic acetylcholine receptors. Brain Res Bull (1997) 0.78
Fetal thrombotic vasculopathy in the placenta: a thrombophilic connection between pregnancy complications and neonatal thrombosis? Placenta (2004) 0.78
Amygdala kindling-induced seizures selectively impair spatial memory. 1. Behavioral characteristics and effects on hippocampal neuronal protein kinase C isoforms. Hippocampus (1992) 0.78
Translocation of protein kinase Cgamma occurs during the early phase of acquisition of food rewarded spatial learning. Behav Neurosci (1998) 0.78
Organotypic suprachiasmatic nuclei cultures of adult voles reflect locomotor behavior: differences in number of vasopressin cells. Chronobiol Int (1999) 0.77
Pre-eclampsia in a woman whose child suffered from lethal carnitine-acylcarnitine translocase deficiency. BJOG (2007) 0.77
Noradrenergic and cholinergic reinnervation of islet grafts in diabetic rats. Cell Transplant (1996) 0.77
Cerebral 1H MR spectroscopy showing elevation of brain guanidinoacetate in argininosuccinate lyase deficiency. Mol Genet Metab (2005) 0.77
End-stage liver disease as the only consequence of a mitochondrial respiratory chain deficiency: no contra-indication for liver transplantation. Eur J Pediatr (2000) 0.77
Long-term cholinergic denervation caused by early postnatal AF64A lesion prevents development of muscarinic receptors in rat hippocampus. J Chem Neuroanat (1992) 0.76
Immunocytochemical demonstration of developmental distribution of muscarinic acetylcholine receptors in rat parietal cortex. Brain Res Dev Brain Res (1995) 0.76
Pilot use of the early motor repertoire in infants with inborn errors of metabolism: outcomes in early and middle childhood. Early Hum Dev (2009) 0.76
Biochemical Monitoring and Management During Pregnancy in Patients with Isovaleric Acidaemia is Helpful to Prevent Metabolic Decompensation. JIMD Rep (2011) 0.76
Phenylketonuria in The Netherlands: 93% of the mutations are detected by single-strand conformation analysis. Hum Hered (1996) 0.76
Predictors of mean phenylalanine levels during the first five years of life in patients with phenylketonuria who were treated early. Dutch National PKU Steering Committee. Acta Paediatr Suppl (1994) 0.75
[Neonatal screening for metabolic diseases: need for efficacy studies]. Ned Tijdschr Geneeskd (2008) 0.75
Amygdala kindling-induced seizures selectively impair spatial memory. 2. Effects on hippocampal neuronal and glial muscarinic acetylcholine receptor. Hippocampus (1992) 0.75
[A child with dark discoloration of urine]. Ned Tijdschr Geneeskd (1999) 0.75
Reversed alterations of hippocampal parvalbumin and protein kinase C-gamma immunoreactivity after stroke in spontaneously hypertensive stroke-prone rats. Stroke (1993) 0.75
Concurrent decrease of vasopressin and protein kinase Calpha immunoreactivity during the light phase in the vole suprachiasmatic nucleus. Neurosci Lett (1998) 0.75
Final commentary: a new chapter. Mol Genet Metab (2010) 0.75
Cerebral 1H MR spectroscopy revealing white matter NAA decreases in glutaric aciduria type I. Mol Genet Metab (2006) 0.75
Partial hypoxanthine-guanine phosphoribosyl transferase deficiency without elevated urinary hypoxanthine excretion. Mol Genet Metab (2006) 0.75
Muscular glycogen storage diseases without increased glycogen content on histopathological examination. Mol Genet Metab (2007) 0.75
Corticosterone modifies muscarinic receptor immunoreactivity in rat hippocampus. Neurosci Lett (1999) 0.75
MR spectroscopy and diffusion tensor imaging of the brain in Sjögren-Larsson syndrome. Mol Genet Metab (2009) 0.75
Hereditary tyrosinaemia type I: a long-term study of the relationship between the urinary excretions of succinylacetone and delta-aminolevulinic acid. J Inherit Metab Dis (1993) 0.75