Published in Mol Cell Biochem on January 04, 2015
Disturbance of redox homeostasis as a contributing underlying pathomechanism of brain and liver alterations in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency. J Inherit Metab Dis (2015) 0.84
Management and long-term evolution of a patient with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. Ital J Pediatr (2017) 0.75
Salivary and Urinary Total Antioxidant Capacity as Biomarkers of Oxidative Stress in Humans. Patholog Res Int (2016) 0.75
3-Hydroxy-3-methylglutaric aciduria with bilateral basal ganglia lesion: A case report. Exp Ther Med (2016) 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
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
Mutations, clinical findings and survival estimates in South American patients with X-linked adrenoleukodystrophy. PLoS One (2012) 0.89
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
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
Gamma-hydroxybutyric acid induces oxidative stress in cerebral cortex of young rats. Neurochem Int (2006) 0.89
The effect of Lorenzo's oil on oxidative stress in X-linked adrenoleukodystrophy. J Neurol Sci (2006) 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
L-carnitine blood levels and oxidative stress in treated phenylketonuric patients. Cell Mol Neurobiol (2008) 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
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
Selective screening for organic acidemias by urine organic acid GC-MS analysis in Brazil: fifteen-year experience. Clin Chim Acta (2008) 0.87
Oxidative stress in phenylketonuria: what is the evidence? Cell Mol Neurobiol (2011) 0.86
Isovaleric acid reduces Na+, K+-ATPase activity in synaptic membranes from cerebral cortex of young rats. Cell Mol Neurobiol (2007) 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
Acute intrastriatal administration of quinolinic acid provokes hyperphosphorylation of cytoskeletal intermediate filament proteins in astrocytes and neurons of rats. Exp Neurol (2010) 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
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
Intracerebroventricular administration of N-acetylaspartic acid impairs antioxidant defenses and promotes protein oxidation in cerebral cortex of rats. Metab Brain Dis (2009) 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
Inhibition of the mitochondrial respiratory chain by phenylalanine in rat cerebral cortex. Neurochem Res (2002) 0.85
Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. Int J Dev Neurosci (2003) 0.84
Ascorbic acid prevents cognitive deficits caused by chronic administration of propionic acid to rats in the water maze. Pharmacol Biochem Behav (2002) 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
Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brain. Neurochem Int (2010) 0.84
Guanosine enhances glutamate uptake in brain cortical slices at normal and excitotoxic conditions. Cell Mol Neurobiol (2002) 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
Impairment of energy metabolism in hippocampus of rats subjected to chemically-induced hyperhomocysteinemia. Biochim Biophys Acta (2003) 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
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
Reduction of butyrylcholinesterase activity in rat serum subjected to hyperhomocysteinemia. Metab Brain Dis (2005) 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 Na(+),K(+)-ATPase activity in hippocampus of rats subjected to chemically induced hyperhomocysteinemia. Neurochem Res (2002) 0.82
Induction of lipid peroxidation and decrease of antioxidant defenses in symptomatic and asymptomatic patients with X-linked adrenoleukodystrophy. Int J Dev Neurosci (2007) 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
Stimulation of lipid peroxidation in vitro in rat brain by the metabolites accumulating in maple syrup urine disease. Metab Brain Dis (2002) 0.82
Proline impairs energy metabolism in cerebral cortex of young rats. Metab Brain Dis (2010) 0.82
Protein and lipid oxidative damage in streptozotocin-induced diabetic rats submitted to forced swimming test: the insulin and clonazepam effect. Metab Brain Dis (2010) 0.82
Proline reduces acetylcholinesterase activity in cerebral cortex of rats. Metab Brain Dis (2003) 0.82
Inhibition of creatine kinase activity in vitro by ethylmalonic acid in cerebral cortex of young rats. Neurochem Res (2002) 0.82
Proline induces oxidative stress in cerebral cortex of rats. Int J Dev Neurosci (2003) 0.82
Glutaric acid induces oxidative stress in brain of young rats. Brain Res (2003) 0.82
Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia. J Bioenerg Biomembr (2011) 0.82
Neurological damage in MSUD: the role of oxidative stress. Cell Mol Neurobiol (2013) 0.82
Inhibition of Na(+),K(+)-ATPase activity in hippocampus of rats subjected to acute administration of homocysteine is prevented by vitamins E and C treatment. Neurochem Res (2002) 0.82
Neurochemical evidence that lysine inhibits synaptic Na+,K+-ATPase activity and provokes oxidative damage in striatum of young rats in vivo. Neurochem Res (2010) 0.81
Hyperphenylalaninemia reduces creatine kinase activity in the cerebral cortex of rats. Int J Dev Neurosci (2003) 0.81
Promotion of lipid and protein oxidative damage in rat brain by ethylmalonic acid. Neurochem Res (2009) 0.81
Oxidative stress in plasma from maple syrup urine disease patients during treatment. Metab Brain Dis (2007) 0.81
Effects of cysteamine on oxidative status in cerebral cortex of rats. Metab Brain Dis (2007) 0.81
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
Lysine induces lipid and protein damage and decreases reduced glutathione concentrations in brain of young rats. Int J Dev Neurosci (2008) 0.81
Induction of oxidative stress in brain of glutaryl-CoA dehydrogenase deficient mice by acute lysine administration. Mol Genet Metab (2012) 0.81
In vitro effects of D-2-hydroxyglutaric acid on glutamate binding, uptake and release in cerebral cortex of rats. J Neurol Sci (2004) 0.81
Evidence that the major metabolites accumulating in medium-chain acyl-CoA dehydrogenase deficiency disturb mitochondrial energy homeostasis in rat brain. Brain Res (2009) 0.81
Inhibition of energy metabolism in cerebral cortex of young rats by the medium-chain fatty acids accumulating in MCAD deficiency. Brain Res (2004) 0.81
Acute administration of 5-oxoproline induces oxidative damage to lipids and proteins and impairs antioxidant defenses in cerebral cortex and cerebellum of young rats. Metab Brain Dis (2010) 0.81
Oxidative stress in Niemann-Pick type C patients: a protective role of N-butyl-deoxynojirimycin therapy. Int J Dev Neurosci (2012) 0.81
Evidence that 2-methylacetoacetate induces oxidative stress in rat brain. Metab Brain Dis (2010) 0.81
Experimental evidence of oxidative stress in plasma of homocystinuric patients: a possible role for homocysteine. Mol Genet Metab (2011) 0.80
Amino acids levels and lipid peroxidation in maple syrup urine disease patients. Clin Biochem (2008) 0.80
Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation. Mol Genet Metab (2012) 0.80
Evidence that folic acid deficiency is a major determinant of hyperhomocysteinemia in Parkinson's disease. Metab Brain Dis (2009) 0.80
Ethylmalonic acid inhibits mitochondrial creatine kinase activity from cerebral cortex of young rats in vitro. Neurochem Res (2003) 0.80
Inhibition of creatine kinase activity from rat cerebral cortex by D-2-hydroxyglutaric acid in vitro. Neurochem Int (2004) 0.80
Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats. Life Sci (2008) 0.80
Promotion of oxidative stress by L-tryptophan in cerebral cortex of rats. Neurochem Int (2006) 0.80