Published in Int J Dev Neurosci on January 25, 2010
Advances and challenges in the treatment of branched-chain amino/keto acid metabolic defects. J Inherit Metab Dis (2011) 0.98
Oxidative stress in phenylketonuria: what is the evidence? Cell Mol Neurobiol (2011) 0.86
Evidence that L-carnitine and selenium supplementation reduces oxidative stress in phenylketonuric patients. Cell Mol Neurobiol (2010) 0.85
Neurological damage in MSUD: the role of oxidative stress. Cell Mol Neurobiol (2013) 0.82
Experimental evidence that methylmalonic acid provokes oxidative damage and compromises antioxidant defenses in nerve terminal and striatum of young rats. Cell Mol Neurobiol (2011) 0.81
Oxidative stress parameters in urine from patients with disorders of propionate metabolism: a beneficial effect of L:-carnitine supplementation. Cell Mol Neurobiol (2011) 0.79
Selected biomarkers as predictive tools in testing efficacy of melatonin and coenzyme Q on propionic acid - induced neurotoxicity in rodent model of autism. BMC Neurosci (2014) 0.76
Oxidative profile exhibited by Mucopolysaccharidosis type IVA patients at diagnosis: Increased keratan urinary levels. Mol Genet Metab Rep (2017) 0.75
Oxidative Stress in Patients with X-Linked Adrenoleukodystrophy. Cell Mol Neurobiol (2015) 0.75
L-Carnitine and Acetyl-L-carnitine Roles and Neuroprotection in Developing Brain. Neurochem Res (2017) 0.75
D-2-hydroxyglutaric acid induces oxidative stress in cerebral cortex of young rats. Eur J Neurosci (2003) 2.62
Natural history, outcome, and treatment efficacy in children and adults with glutaryl-CoA dehydrogenase deficiency. Pediatr Res (2006) 2.31
Antioxidant effect of cysteamine in brain cortex of young rats. Neurochem Res (2007) 1.59
Homocysteine and other markers of cardiovascular risk during a manic episode in patients with bipolar disorder. Rev Bras Psiquiatr (2013) 1.45
Mitochondrial energy metabolism is markedly impaired by D-2-hydroxyglutaric acid in rat tissues. Mol Genet Metab (2005) 1.32
Induction of oxidative stress by L-2-hydroxyglutaric acid in rat brain. J Neurosci Res (2003) 1.25
An overview of L-2-hydroxyglutarate dehydrogenase gene (L2HGDH) variants: a genotype-phenotype study. Hum Mutat (2010) 1.11
Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease. Biochim Biophys Acta (2003) 1.11
L-2-Hydroxyglutaric aciduria: pattern of MR imaging abnormalities in 56 patients. Radiology (2009) 1.04
Resveratrol protects C6 astrocyte cell line against hydrogen peroxide-induced oxidative stress through heme oxygenase 1. PLoS One (2013) 1.02
Globotriaosylceramide is correlated with oxidative stress and inflammation in Fabry patients treated with enzyme replacement therapy. Biochim Biophys Acta (2011) 0.98
L-2-hydroxyglutaric acid inhibits mitochondrial creatine kinase activity from cerebellum of developing rats. Int J Dev Neurosci (2003) 0.96
Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain. Brain Res (2010) 0.96
Hexacosanoic and docosanoic acids plasma levels in patients with cerebral childhood and asymptomatic X-linked adrenoleukodystrophy: Lorenzo's oil effect. Metab Brain Dis (2007) 0.93
In vitro evidence for an antioxidant role of 3-hydroxykynurenine and 3-hydroxyanthranilic acid in the brain. Neurochem Int (2006) 0.93
L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders. Gene (2013) 0.92
Inhibition of brain energy metabolism by the branched-chain amino acids accumulating in maple syrup urine disease. Neurochem Res (2007) 0.91
alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats. Metab Brain Dis (2005) 0.89
Alpha-lipoic acid modifies oxidative stress parameters in sickle cell trait subjects and sickle cell patients. Clin Nutr (2009) 0.89
Gamma-hydroxybutyric acid induces oxidative stress in cerebral cortex of young rats. Neurochem Int (2006) 0.89
Medium-chain fatty acids accumulating in MCAD deficiency elicit lipid and protein oxidative damage and decrease non-enzymatic antioxidant defenses in rat brain. Neurochem Int (2009) 0.89
Early biochemical effects after unilateral hypoxia-ischemia in the immature rat brain. Int J Dev Neurosci (2011) 0.88
The effect of Lorenzo's oil on oxidative stress in X-linked adrenoleukodystrophy. J Neurol Sci (2006) 0.87
L-carnitine blood levels and oxidative stress in treated phenylketonuric patients. Cell Mol Neurobiol (2008) 0.87
Morphological alterations and induction of oxidative stress in glial cells caused by the branched-chain alpha-keto acids accumulating in maple syrup urine disease. Neurochem Int (2006) 0.87
Marked reduction of Na(+), K(+)-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice. Mol Genet Metab (2012) 0.87
Inhibition of cytochrome c oxidase activity in rat cerebral cortex and human skeletal muscle by D-2-hydroxyglutaric acid in vitro. Biochim Biophys Acta (2002) 0.87
Development of an animal model for chronic mild hyperhomocysteinemia and its response to oxidative damage. Int J Dev Neurosci (2011) 0.87
Oxidative stress in phenylketonuria: what is the evidence? Cell Mol Neurobiol (2011) 0.86
Acute intrastriatal administration of quinolinic acid provokes hyperphosphorylation of cytoskeletal intermediate filament proteins in astrocytes and neurons of rats. Exp Neurol (2010) 0.86
Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues. Int J Dev Neurosci (2006) 0.86
Effects of L-2-hydroxyglutaric acid on various parameters of the glutamatergic system in cerebral cortex of rats. Metab Brain Dis (2003) 0.86
Isovaleric acid reduces Na+, K+-ATPase activity in synaptic membranes from cerebral cortex of young rats. Cell Mol Neurobiol (2007) 0.86
Quinolinic acid reduces the antioxidant defenses in cerebral cortex of young rats. Int J Dev Neurosci (2005) 0.86
Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease. Metab Brain Dis (2006) 0.85
Induction of oxidative stress by the metabolites accumulating in isovaleric acidemia in brain cortex of young rats. Free Radic Res (2008) 0.85
Intracerebroventricular administration of N-acetylaspartic acid impairs antioxidant defenses and promotes protein oxidation in cerebral cortex of rats. Metab Brain Dis (2009) 0.85
Increased oxidative damage in carriers of the germline TP53 p.R337H mutation. PLoS One (2012) 0.85
In vivo and in vitro effects of proline on some parameters of oxidative stress in rat brain. Brain Res (2003) 0.85
Inhibition of the mitochondrial respiratory chain by phenylalanine in rat cerebral cortex. Neurochem Res (2002) 0.85
Ascorbic acid prevents cognitive deficits caused by chronic administration of propionic acid to rats in the water maze. Pharmacol Biochem Behav (2002) 0.84
Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. Int J Dev Neurosci (2003) 0.84
Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria. Mol Cell Biochem (2015) 0.84
Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brain. Neurochem Int (2010) 0.84
Chronic variable stress induces oxidative stress and decreases butyrylcholinesterase activity in blood of rats. J Neural Transm (Vienna) (2010) 0.84
Effect of the branched-chain alpha-keto acids accumulating in maple syrup urine disease on S100B release from glial cells. J Neurol Sci (2007) 0.84
Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem (2007) 0.84
Guanosine enhances glutamate uptake in brain cortical slices at normal and excitotoxic conditions. Cell Mol Neurobiol (2002) 0.84
Oxidative stress in patients with mucopolysaccharidosis type II before and during enzyme replacement therapy. Mol Genet Metab (2011) 0.84
In vitro effect of homocysteine on some parameters of oxidative stress in rat hippocampus. Metab Brain Dis (2003) 0.84
Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice. Life Sci (2012) 0.83
Guanidinoacetate decreases antioxidant defenses and total protein sulfhydryl content in striatum of rats. Neurochem Res (2008) 0.83
Inhibition of the electron transport chain and creatine kinase activity by ethylmalonic acid in human skeletal muscle. Metab Brain Dis (2006) 0.83
Citrulline and ammonia accumulating in citrullinemia reduces antioxidant capacity of rat brain in vitro. Metab Brain Dis (2006) 0.83
Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Brain Res (2010) 0.83
Experimental hyperphenylalaninemia provokes oxidative stress in rat brain. Biochim Biophys Acta (2002) 0.83
Impairment of energy metabolism in hippocampus of rats subjected to chemically-induced hyperhomocysteinemia. Biochim Biophys Acta (2003) 0.83
Effect of short- and long-term exposition to high phenylalanine blood levels on oxidative damage in phenylketonuric patients. Int J Dev Neurosci (2009) 0.83
DNA damage in leukocytes from pretreatment mucopolysaccharidosis type II patients; protective effect of enzyme replacement therapy. Mutat Res (2011) 0.83
Evidence for a synergistic action of glutaric and 3-hydroxyglutaric acids disturbing rat brain energy metabolism. Int J Dev Neurosci (2007) 0.83
Reduction of butyrylcholinesterase activity in rat serum subjected to hyperhomocysteinemia. Metab Brain Dis (2005) 0.83
Reduction of Na(+),K(+)-ATPase activity in hippocampus of rats subjected to chemically induced hyperhomocysteinemia. Neurochem Res (2002) 0.82
Prevention by L-carnitine of DNA damage induced by propionic and L-methylmalonic acids in human peripheral leukocytes in vitro. Mutat Res (2010) 0.82