Published in Life Sci on January 11, 2008
Inherited mitochondrial optic neuropathies. J Med Genet (2008) 2.50
The 3-methylglutaconic acidurias: what's new? J Inherit Metab Dis (2010) 1.08
Oxidative stress in phenylketonuria: what is the evidence? Cell Mol Neurobiol (2011) 0.86
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
3-Methylglutaconyl-Coenzyme-A Hydratase Deficiency and the Development of Dilated Cardiomyopathy. Cardiol Res (2014) 0.75
Neurochemical evidence that the metabolites accumulating in 3-methylcrotonyl-CoA carboxylase deficiency induce oxidative damage in cerebral cortex of young rats. Cell Mol Neurobiol (2012) 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
Hyperhomocysteinemia reduces glutamate uptake in parietal cortex of rats. Int J Dev Neurosci (2009) 1.43
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
Effects of low-power laser irradiation (LPLI) at different wavelengths and doses on oxidative stress and fibrogenesis parameters in an animal model of wound healing. Lasers Med Sci (2010) 1.16
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
Short bouts of mild-intensity physical exercise improve spatial learning and memory in aging rats: involvement of hippocampal plasticity via AKT, CREB and BDNF signaling. Mech Ageing Dev (2011) 1.05
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
Homocysteine induces oxidative stress, inflammatory infiltration, fibrosis and reduces glycogen/glycoprotein content in liver of rats. Int J Dev Neurosci (2009) 1.00
NTPDase and 5'-nucleotidase activities in physiological and disease conditions: new perspectives for human health. Biofactors (2007) 0.99
Homocysteine induces oxidative-nitrative stress in heart of rats: prevention by folic acid. Cardiovasc Toxicol (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
Alterations in architecture and metabolism induced by ultraviolet radiation-B in the carragenophyte Chondracanthus teedei (Rhodophyta, Gigartinales). Protoplasma (2011) 0.95
Neurobiological alterations induced by exercise and their impact on depressive disorders [corrected]. Clin Pract Epidemiol Ment Health (2010) 0.94
Vitamins E and C pretreatment prevents ovariectomy-induced memory deficits in water maze. Neurobiol Learn Mem (2005) 0.93
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
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
Effects of inorganic selenium administration in methylmercury-induced neurotoxicity in mouse cerebral cortex. Int J Dev Neurosci (2010) 0.89
Chronic hyperhomocysteinemia alters antioxidant defenses and increases DNA damage in brain and blood of rats: protective effect of folic acid. Neurochem Int (2008) 0.88
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
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
Development of an animal model for chronic mild hyperhomocysteinemia and its response to oxidative damage. Int J Dev Neurosci (2011) 0.87
The intranasal administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP): a new rodent model to test palliative and neuroprotective agents for Parkinson's disease. Curr Pharm Des (2011) 0.87
Methylphenidate affects memory, brain-derived neurotrophic factor immunocontent and brain acetylcholinesterase activity in the rat. Neurobiol Learn Mem (2010) 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
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
Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues. Int J Dev Neurosci (2006) 0.86
Resveratrol prevents oxidative stress and inhibition of Na(+)K(+)-ATPase activity induced by transient global cerebral ischemia in rats. J Nutr Biochem (2011) 0.86
Quinolinic acid reduces the antioxidant defenses in cerebral cortex of young rats. Int J Dev Neurosci (2005) 0.86
Chronic administration of branched-chain amino acids impairs spatial memory and increases brain-derived neurotrophic factor in a rat model. J Inherit Metab Dis (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
Induction of oxidative stress by the metabolites accumulating in isovaleric acidemia in brain cortex of young rats. Free Radic Res (2008) 0.85
Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease. Metab Brain Dis (2006) 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
Inhibition of the mitochondrial respiratory chain by phenylalanine in rat cerebral cortex. Neurochem Res (2002) 0.85
Pretreatment with vitamins E and C prevent the impairment of memory caused by homocysteine administration in rats. Metab Brain Dis (2002) 0.85
Neonatal hypoxia-ischemia induces sex-related changes in rat brain mitochondria. Mitochondrion (2011) 0.84
Guanosine enhances glutamate uptake in brain cortical slices at normal and excitotoxic conditions. Cell Mol Neurobiol (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
Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. Int J Dev Neurosci (2003) 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
Proanthocyanidin-rich fraction from Croton celtidifolius Baill confers neuroprotection in the intranasal 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine rat model of Parkinson's disease. J Neural Transm (Vienna) (2010) 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
Chronic variable stress induces oxidative stress and decreases butyrylcholinesterase activity in blood of rats. J Neural Transm (Vienna) (2010) 0.84
Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem (2007) 0.84
Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria. Mol Cell Biochem (2015) 0.84
The influence of low-level laser therapy on parameters of oxidative stress and DNA damage on muscle and plasma in rats with heart failure. Lasers Med Sci (2014) 0.84
The janus face of resveratrol in astroglial cells. Neurotox Res (2009) 0.84
Homocysteine induces energy imbalance in rat skeletal muscle: is creatine a protector? Cell Biochem Funct (2012) 0.84