PubRank

Moacir Wajner

Author PubWeight™ 163.60‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 D-2-hydroxyglutaric acid induces oxidative stress in cerebral cortex of young rats. Eur J Neurosci 2003 2.62
2 Natural history, outcome, and treatment efficacy in children and adults with glutaryl-CoA dehydrogenase deficiency. Pediatr Res 2006 2.31
3 Antioxidant effect of cysteamine in brain cortex of young rats. Neurochem Res 2007 1.59
4 Mitochondrial energy metabolism is markedly impaired by D-2-hydroxyglutaric acid in rat tissues. Mol Genet Metab 2005 1.32
5 Induction of oxidative stress by L-2-hydroxyglutaric acid in rat brain. J Neurosci Res 2003 1.25
6 An overview of L-2-hydroxyglutarate dehydrogenase gene (L2HGDH) variants: a genotype-phenotype study. Hum Mutat 2010 1.11
7 Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease. Biochim Biophys Acta 2003 1.11
8 L-2-Hydroxyglutaric aciduria: pattern of MR imaging abnormalities in 56 patients. Radiology 2009 1.04
9 Resveratrol protects C6 astrocyte cell line against hydrogen peroxide-induced oxidative stress through heme oxygenase 1. PLoS One 2013 1.02
10 L-2-hydroxyglutaric acid inhibits mitochondrial creatine kinase activity from cerebellum of developing rats. Int J Dev Neurosci 2003 0.96
11 Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain. Brain Res 2010 0.96
12 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
13 In vitro evidence for an antioxidant role of 3-hydroxykynurenine and 3-hydroxyanthranilic acid in the brain. Neurochem Int 2006 0.93
14 L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders. Gene 2013 0.92
15 Inhibition of brain energy metabolism by the branched-chain amino acids accumulating in maple syrup urine disease. Neurochem Res 2007 0.91
16 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
17 Gamma-hydroxybutyric acid induces oxidative stress in cerebral cortex of young rats. Neurochem Int 2006 0.89
18 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
19 The effect of Lorenzo's oil on oxidative stress in X-linked adrenoleukodystrophy. J Neurol Sci 2006 0.87
20 L-carnitine blood levels and oxidative stress in treated phenylketonuric patients. Cell Mol Neurobiol 2008 0.87
21 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
22 Evidence that the branched-chain alpha-keto acids accumulating in maple syrup urine disease induce morphological alterations and death in cultured astrocytes from rat cerebral cortex. Glia 2004 0.87
23 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
24 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
25 Oxidative stress in phenylketonuria: what is the evidence? Cell Mol Neurobiol 2011 0.86
26 Acute intrastriatal administration of quinolinic acid provokes hyperphosphorylation of cytoskeletal intermediate filament proteins in astrocytes and neurons of rats. Exp Neurol 2010 0.86
27 Effects of L-2-hydroxyglutaric acid on various parameters of the glutamatergic system in cerebral cortex of rats. Metab Brain Dis 2003 0.86
28 Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues. Int J Dev Neurosci 2006 0.86
29 Isovaleric acid reduces Na+, K+-ATPase activity in synaptic membranes from cerebral cortex of young rats. Cell Mol Neurobiol 2007 0.86
30 Quinolinic acid reduces the antioxidant defenses in cerebral cortex of young rats. Int J Dev Neurosci 2005 0.86
31 Intracerebroventricular administration of N-acetylaspartic acid impairs antioxidant defenses and promotes protein oxidation in cerebral cortex of rats. Metab Brain Dis 2009 0.85
32 Induction of oxidative stress by the metabolites accumulating in isovaleric acidemia in brain cortex of young rats. Free Radic Res 2008 0.85
33 In vivo and in vitro effects of proline on some parameters of oxidative stress in rat brain. Brain Res 2003 0.85
34 Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease. Metab Brain Dis 2006 0.85
35 Inhibition of the mitochondrial respiratory chain by phenylalanine in rat cerebral cortex. Neurochem Res 2002 0.85
36 Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem 2007 0.84
37 Guanosine enhances glutamate uptake in brain cortical slices at normal and excitotoxic conditions. Cell Mol Neurobiol 2002 0.84
38 Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brain. Neurochem Int 2010 0.84
39 Ascorbic acid prevents cognitive deficits caused by chronic administration of propionic acid to rats in the water maze. Pharmacol Biochem Behav 2002 0.84
40 Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. Int J Dev Neurosci 2003 0.84
41 Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria. Mol Cell Biochem 2015 0.84
42 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
43 In vitro effect of homocysteine on some parameters of oxidative stress in rat hippocampus. Metab Brain Dis 2003 0.84
44 Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice. Life Sci 2012 0.83
45 Experimental hyperphenylalaninemia provokes oxidative stress in rat brain. Biochim Biophys Acta 2002 0.83
46 Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Brain Res 2010 0.83
47 Guanidinoacetate decreases antioxidant defenses and total protein sulfhydryl content in striatum of rats. Neurochem Res 2008 0.83
48 Impairment of energy metabolism in hippocampus of rats subjected to chemically-induced hyperhomocysteinemia. Biochim Biophys Acta 2003 0.83
49 Inhibition of the electron transport chain and creatine kinase activity by ethylmalonic acid in human skeletal muscle. Metab Brain Dis 2006 0.83
50 Citrulline and ammonia accumulating in citrullinemia reduces antioxidant capacity of rat brain in vitro. Metab Brain Dis 2006 0.83
51 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
52 Reduction of butyrylcholinesterase activity in rat serum subjected to hyperhomocysteinemia. Metab Brain Dis 2005 0.83
53 Evidence for a synergistic action of glutaric and 3-hydroxyglutaric acids disturbing rat brain energy metabolism. Int J Dev Neurosci 2007 0.83
54 Reduction of Na(+),K(+)-ATPase activity in hippocampus of rats subjected to chemically induced hyperhomocysteinemia. Neurochem Res 2002 0.82
55 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
56 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
57 Stimulation of lipid peroxidation in vitro in rat brain by the metabolites accumulating in maple syrup urine disease. Metab Brain Dis 2002 0.82
58 Proline reduces acetylcholinesterase activity in cerebral cortex of rats. Metab Brain Dis 2003 0.82
59 Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia. J Bioenerg Biomembr 2011 0.82
60 Proline impairs energy metabolism in cerebral cortex of young rats. Metab Brain Dis 2010 0.82
61 Proline induces oxidative stress in cerebral cortex of rats. Int J Dev Neurosci 2003 0.82
62 Neurological damage in MSUD: the role of oxidative stress. Cell Mol Neurobiol 2013 0.82
63 Glutaric acid induces oxidative stress in brain of young rats. Brain Res 2003 0.82
64 Inhibition of creatine kinase activity in vitro by ethylmalonic acid in cerebral cortex of young rats. Neurochem Res 2002 0.82
65 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
66 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
67 Hyperphenylalaninemia reduces creatine kinase activity in the cerebral cortex of rats. Int J Dev Neurosci 2003 0.81
68 Lysine induces lipid and protein damage and decreases reduced glutathione concentrations in brain of young rats. Int J Dev Neurosci 2008 0.81
69 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
70 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
71 Oxidative stress in Niemann-Pick type C patients: a protective role of N-butyl-deoxynojirimycin therapy. Int J Dev Neurosci 2012 0.81
72 Induction of oxidative stress in brain of glutaryl-CoA dehydrogenase deficient mice by acute lysine administration. Mol Genet Metab 2012 0.81
73 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
74 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
75 Promotion of lipid and protein oxidative damage in rat brain by ethylmalonic acid. Neurochem Res 2009 0.81
76 Oxidative stress in plasma from maple syrup urine disease patients during treatment. Metab Brain Dis 2007 0.81
77 Effects of cysteamine on oxidative status in cerebral cortex of rats. Metab Brain Dis 2007 0.81
78 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
79 Evidence that 2-methylacetoacetate induces oxidative stress in rat brain. Metab Brain Dis 2010 0.81
80 Experimental evidence of oxidative stress in plasma of homocystinuric patients: a possible role for homocysteine. Mol Genet Metab 2011 0.80
81 Amino acids levels and lipid peroxidation in maple syrup urine disease patients. Clin Biochem 2008 0.80
82 Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation. Mol Genet Metab 2012 0.80
83 Glutaric acid administration impairs energy metabolism in midbrain and skeletal muscle of young rats. Neurochem Res 2005 0.80
84 Ethylmalonic acid inhibits mitochondrial creatine kinase activity from cerebral cortex of young rats in vitro. Neurochem Res 2003 0.80
85 Induction of oxidative stress by chronic and acute glutaric acid administration to rats. Cell Mol Neurobiol 2007 0.80
86 Evidence that folic acid deficiency is a major determinant of hyperhomocysteinemia in Parkinson's disease. Metab Brain Dis 2009 0.80
87 Evidence that glutaric acid reduces glutamate uptake by cerebral cortex of infant rats. Life Sci 2007 0.80
88 Lower in vivo brain extracellular GABA concentration in diabetic rats during forced swimming. Brain Res 2003 0.80
89 Experimental evidence that phenylalanine provokes oxidative stress in hippocampus and cerebral cortex of developing rats. Cell Mol Neurobiol 2009 0.80
90 Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats. Life Sci 2008 0.80
91 Inhibition of creatine kinase activity from rat cerebral cortex by D-2-hydroxyglutaric acid in vitro. Neurochem Int 2004 0.80
92 The effects of the interactions between amino acids on pyruvate kinase activity from the brain cortex of young rats. Int J Dev Neurosci 2005 0.80
93 Effects of 1,4-butanediol administration on oxidative stress in rat brain: study of the neurotoxicity of gamma-hydroxybutyric acid in vivo. Metab Brain Dis 2009 0.80
94 Promotion of oxidative stress by L-tryptophan in cerebral cortex of rats. Neurochem Int 2006 0.80
95 Tryptophan administration induces oxidative stress in brain cortex of rats. Metab Brain Dis 2008 0.80
96 Tyrosine promotes oxidative stress in cerebral cortex of young rats. Int J Dev Neurosci 2008 0.80
97 3-Hydroxyglutaric acid induces oxidative stress and decreases the antioxidant defenses in cerebral cortex of young rats. Brain Res 2002 0.80
98 Brain energy metabolism is compromised by the metabolites accumulating in homocystinuria. Neurochem Int 2003 0.79
99 Chemically induced model of hypermethioninemia in rats. J Neurosci Methods 2006 0.79
100 Alanine prevents the in vitro inhibition of glycolysis caused by phenylalanine in brain cortex of rats. Metab Brain Dis 2003 0.79
101 Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver. Int J Dev Neurosci 2009 0.79
102 Toxicity of octanoate and decanoate in rat peripheral tissues: evidence of bioenergetic dysfunction and oxidative damage induction in liver and skeletal muscle. Mol Cell Biochem 2011 0.79
103 In vitro phosphorylation of cytoskeletal proteins from cerebral cortex of rats. Brain Res Brain Res Protoc 2003 0.79
104 Marked inhibition of Na+, K(+)- ATPase activity and the respiratory chain by phytanic acid in cerebellum from young rats: possible underlying mechanisms of cerebellar ataxia in Refsum disease. J Bioenerg Biomembr 2012 0.79
105 Folic acid pretreatment prevents the reduction of Na(+),K(+)-ATPase and butyrylcholinesterase activities in rats subjected to acute hyperhomocysteinemia. Int J Dev Neurosci 2006 0.79
106 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
107 Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain. Life Sci 2010 0.79
108 Chronic postnatal ornithine administration to rats provokes learning deficit in the open field task. Metab Brain Dis 2012 0.79
109 Disturbance of brain energy and redox homeostasis provoked by sulfite and thiosulfate: potential pathomechanisms involved in the neuropathology of sulfite oxidase deficiency. Gene 2013 0.79
110 Morphological alterations and cell death provoked by the branched-chain alpha-amino acids accumulating in maple syrup urine disease in astrocytes from rat cerebral cortex. Cell Mol Neurobiol 2005 0.79
111 Propionic acid induces cytoskeletal alterations in cultured astrocytes from rat cerebral cortex. Metab Brain Dis 2006 0.79
112 In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: a potential pathophysiological mechanism of striatal damage in this disorder. Mol Genet Metab 2013 0.79
113 Impairment of brain redox homeostasis caused by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome in vivo. Metab Brain Dis 2012 0.79
114 Intrahippocampal administration of the branched-chain alpha-hydroxy acids accumulating in maple syrup urine disease compromises rat performance in aversive and non-aversive behavioral tasks. J Neurol Sci 2005 0.78
115 Homocysteine inhibits butyrylcholinesterase activity in rat serum. Metab Brain Dis 2003 0.78
116 In vitro evidence that D-serine disturbs the citric acid cycle through inhibition of citrate synthase activity in rat cerebral cortex. Brain Res 2009 0.78
117 Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I. Metab Brain Dis 2010 0.78
118 Alpha-tocopherol and ascorbic acid administration prevents the impairment of brain energy metabolism of hyperargininemic rats. Cell Mol Neurobiol 2006 0.78
119 Age and brain structural related effects of glutaric and 3-hydroxyglutaric acids on glutamate binding to plasma membranes during rat brain development. Cell Mol Neurobiol 2007 0.78
120 Evidence that 3-hydroxyglutaric acid interacts with NMDA receptors in synaptic plasma membranes from cerebral cortex of young rats. Neurochem Int 2004 0.78
121 Signaling mechanisms downstream of quinolinic acid targeting the cytoskeleton of rat striatal neurons and astrocytes. Exp Neurol 2011 0.78
122 Mitochondrial permeability transition in neuronal damage promoted by Ca2+ and respiratory chain complex II inhibition. J Neurochem 2004 0.78
123 Inhibition of pyruvate kinase activity by cystine in brain cortex of rats. Brain Res 2004 0.78
124 Glutaric acid moderately compromises energy metabolism in rat brain. Int J Dev Neurosci 2005 0.78
125 N-acetylaspartic acid promotes oxidative stress in cerebral cortex of rats. Int J Dev Neurosci 2007 0.78
126 Astrocytic proliferation and mitochondrial dysfunction induced by accumulated glutaric acidemia I (GAI) metabolites: possible implications for GAI pathogenesis. Neurobiol Dis 2008 0.78
127 Evidence that oxidative stress is involved in the inhibitory effect of proline on Na(+),K(+)-ATPase activity in synaptic plasma membrane of rat hippocampus. Int J Dev Neurosci 2003 0.78
128 3-Methylcrotonylglycine disrupts mitochondrial energy homeostasis and inhibits synaptic Na(+),K (+)-ATPase activity in brain of young rats. Cell Mol Neurobiol 2011 0.78
129 Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria. J Bioenerg Biomembr 2012 0.78
130 Kynurenines impair energy metabolism in rat cerebral cortex. Cell Mol Neurobiol 2006 0.78
131 Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of L-carnitine supplementation. Int J Dev Neurosci 2010 0.78
132 Inhibition of Na+, K+-ATPase activity in rat striatum by the metabolites accumulated in Lesch-Nyhan disease. Int J Dev Neurosci 2004 0.78
133 Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex. Metab Brain Dis 2007 0.78
134 Characterization of the inhibition of pyruvate kinase caused by phenylalanine and phenylpyruvate in rat brain cortex. Brain Res 2003 0.78
135 Chronic treatment with glutaric acid induces partial tolerance to excitotoxicity in neuronal cultures from chick embryo telencephalons. J Neurosci Res 2002 0.78
136 Glutaric acid stimulates glutamate binding and astrocytic uptake and inhibits vesicular glutamate uptake in forebrain from young rats. Neurochem Int 2004 0.78
137 Arginine administration decreases cerebral cortex acetylcholinesterase and serum butyrylcholinesterase probably by oxidative stress induction. Neurochem Res 2004 0.78
138 Ascorbic acid prevents water maze behavioral deficits caused by early postnatal methylmalonic acid administration in the rat. Brain Res 2003 0.78
139 Inhibition of creatine kinase activity by lysine in rat cerebral cortex. Metab Brain Dis 2009 0.78
140 Oxidative stress is induced in female carriers of X-linked adrenoleukodystrophy. J Neurol Sci 2007 0.78
141 Pristanic acid promotes oxidative stress in brain cortex of young rats: a possible pathophysiological mechanism for brain damage in peroxisomal disorders. Brain Res 2011 0.78
142 Inhibition of Na+, K+-ATPase activity by the metabolites accumulating in homocystinuria. Metab Brain Dis 2002 0.78
143 In vitro homocysteine inhibits platelet Na+,K+-ATPase and serum butyrylcholinesterase activities of young rats. Metab Brain Dis 2003 0.78
144 Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect. Int J Dev Neurosci 2012 0.78
145 Inhibition of Na+, K+-ATPase activity in rat striatum by guanidinoacetate. Int J Dev Neurosci 2003 0.78
146 Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation. Metab Brain Dis 2015 0.78
147 Alanine prevents the inhibition of pyruvate kinase activity caused by tryptophan in cerebral cortex of rats. Metab Brain Dis 2003 0.77
148 Tyrosine administration decreases glutathione and stimulates lipid and protein oxidation in rat cerebral cortex. Metab Brain Dis 2009 0.77
149 Creatine kinase activity from rat brain is inhibited by branched-chain amino acids in vitro. Neurochem Res 2003 0.77
150 D-serine induces lipid and protein oxidative damage and decreases glutathione levels in brain cortex of rats. Brain Res 2008 0.77
151 Evaluation of the effect of chronic administration of drugs on rat behavior in the water maze task. Brain Res Brain Res Protoc 2003 0.77
152 Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease. Mol Cell Biochem 2012 0.77
153 Evidence that quinolinic acid severely impairs energy metabolism through activation of NMDA receptors in striatum from developing rats. J Neurochem 2006 0.77
154 Glycine provokes lipid oxidative damage and reduces the antioxidant defenses in brain cortex of young rats. Cell Mol Neurobiol 2008 0.77
155 Creatine administration prevents Na+,K+-ATPase inhibition induced by intracerebroventricular administration of isovaleric acid in cerebral cortex of young rats. Brain Res 2009 0.77
156 Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium-chain acyl-CoA dehydrogenase deficiency. J Inherit Metab Dis 2014 0.77
157 5-Oxoproline reduces non-enzymatic antioxidant defenses in vitro in rat brain. Metab Brain Dis 2007 0.77
158 Glycine intrastriatal administration induces lipid and protein oxidative damage and alters the enzymatic antioxidant defenses in rat brain. Life Sci 2011 0.77
159 Alanine prevents the reduction of pyruvate kinase activity in brain cortex of rats subjected to chemically induced hyperphenylalaninemia. Neurochem Res 2002 0.77
160 Simvastatin treatment prevents oxidative damage to DNA in whole blood leukocytes of dyslipidemic type 2 diabetic patients. Cell Biochem Funct 2010 0.77
161 The molecular landscape of propionic acidemia and methylmalonic aciduria in Latin America. J Inherit Metab Dis 2010 0.77
162 Oxidative stress induction by cis-4-decenoic acid: relevance for MCAD deficiency. Free Radic Res 2007 0.77
163 Neurochemical evidence that glycine induces bioenergetical dysfunction. Neurochem Int 2010 0.77
164 d-Serine administration provokes lipid oxidation and decreases the antioxidant defenses in rat striatum. Int J Dev Neurosci 2010 0.77
165 Inhibition of mitochondrial creatine kinase activity by D-2-hydroxyglutaric acid in cerebellum of young rats. Neurochem Res 2003 0.77
166 Methionine alters Na+,K+-ATPase activity, lipid peroxidation and nonenzymatic antioxidant defenses in rat hippocampus. Int J Dev Neurosci 2005 0.77
167 Arginine administration reduces creatine kinase activity in rat cerebellum. Metab Brain Dis 2007 0.77
168 Effect of hypoxanthine on Na+,K+-ATPase activity and some parameters of oxidative stress in rat striatum. Brain Res 2005 0.77
169 Evidence that 3-hydroxy-3-methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex. J Neurosci Res 2008 0.77
170 Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit. Behav Brain Res 2008 0.77
171 Lipid, Oxidative and Inflammatory Profile and Alterations in the Enzymes Paraoxonase and Butyrylcholinesterase in Plasma of Patients with Homocystinuria Due CBS Deficiency: The Vitamin B12 and Folic Acid Importance. Cell Mol Neurobiol 2015 0.76
172 Intrahippocampal administration of the alpha-keto acids accumulating in maple syrup urine disease provokes learning deficits in rats. Pharmacol Biochem Behav 2004 0.76
173 In vitro effect of quinolinic acid on energy metabolism in brain of young rats. Neurosci Res 2006 0.76
174 alpha-Ketoisocaproic acid regulates phosphorylation of intermediate filaments in postnatal rat cortical slices through ionotropic glutamatergic receptors. Brain Res Dev Brain Res 2002 0.76
175 Ethylmalonic acid induces permeability transition in isolated brain mitochondria. Neurotox Res 2014 0.76
176 Kinetic studies on the inhibition of creatine kinase activity by branched-chain alpha-amino acids in the brain cortex of rats. Int J Dev Neurosci 2003 0.76
177 Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester. Pediatr Nephrol 2007 0.76
178 Neurochemical evidence that 3-methylglutaric acid inhibits synaptic Na+,K+-ATPase activity probably through oxidative damage in brain cortex of young rats. Int J Dev Neurosci 2010 0.76
179 Disruption of redox homeostasis in cerebral cortex of developing rats by acylcarnitines accumulating in medium-chain acyl-CoA dehydrogenase deficiency. Int J Dev Neurosci 2012 0.76
180 Ethylmalonic acid impairs brain mitochondrial succinate and malate transport. Mol Genet Metab 2011 0.76
181 Branched-chain alpha-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells. Metab Brain Dis 2005 0.76
182 Disruption of mitochondrial homeostasis by phytanic acid in cerebellum of young rats. Cerebellum 2013 0.76
183 A chemically-induced acute model of maple syrup urine disease in rats for neurochemical studies. J Neurosci Methods 2006 0.76
184 2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain. Brain Res 2012 0.76
185 Experimental evidence that pristanic acid disrupts mitochondrial homeostasis in brain of young rats. J Neurosci Res 2011 0.76
186 Lipoic acid prevents oxidative stress in vitro and in vivo by an acute hyperphenylalaninemia chemically-induced in rat brain. J Neurol Sci 2010 0.76
187 Alpha-keto-beta-methylvaleric acid increases the in vitro phosphorylation of intermediate filaments in cerebral cortex of young rats through the gabaergic system. J Neurol Sci 2004 0.76
188 Chronic early postnatal glutaric acid administration causes cognitive deficits in the water maze. Behav Brain Res 2007 0.76
189 Energy metabolism is compromised in skeletal muscle of rats chronically-treated with glutaric acid. Metab Brain Dis 2007 0.76
190 On the mechanism of the inhibition of Na(+), K(+)-ATPase activity caused by homocysteine. Int J Dev Neurosci 2002 0.76
191 Reduction of Na+, K+-ATPase activity and expression in cerebral cortex of glutaryl-CoA dehydrogenase deficient mice: a possible mechanism for brain injury in glutaric aciduria type I. Mol Genet Metab 2012 0.76
192 Pyruvate kinase activity and δ-aminolevulinic acid dehydratase activity as biomarkers of toxicity in workers exposed to lead. Arch Environ Contam Toxicol 2012 0.76
193 Evidence that DNA damage is associated to phenylalanine blood levels in leukocytes from phenylketonuric patients. Mutat Res 2009 0.76
194 Neurochemical evidence that phytanic acid induces oxidative damage and reduces the antioxidant defenses in cerebellum and cerebral cortex of rats. Life Sci 2010 0.76
195 Glycine intracerebroventricular administration disrupts mitochondrial energy homeostasis in cerebral cortex and striatum of young rats. Neurotox Res 2013 0.76
196 Effect of leucine administration on creatine kinase activity in rat brain. Metab Brain Dis 2003 0.76
197 Experimental evidence that ornithine and homocitrulline disrupt energy metabolism in brain of young rats. Brain Res 2009 0.76
198 In vitro evidence that phytanic acid compromises Na(+),K(+)-ATPase activity and the electron flow through the respiratory chain in brain cortex from young rats. Brain Res 2010 0.76
199 Evidence that glycine induces lipid peroxidation and decreases glutathione concentrations in rat cerebellum. Mol Cell Biochem 2014 0.76
200 Effect of in vivo administration of ethylmalonic acid on energy metabolism in rat tissues. Metab Brain Dis 2006 0.76
201 Biochemical, histopathological and behavioral alterations caused by intrastriatal administration of quinolic acid to young rats. FEBS J 2014 0.76
202 N-acetylaspartic acid impairs enzymatic antioxidant defenses and enhances hydrogen peroxide concentration in rat brain. Metab Brain Dis 2010 0.76
203 Disturbance of redox homeostasis by ornithine and homocitrulline in rat cerebellum: a possible mechanism of cerebellar dysfunction in HHH syndrome. Life Sci 2013 0.76
204 Evidence that the inhibitory effects of guanidinoacetate on the activities of the respiratory chain, Na+,K+-ATPase and creatine kinase can be differentially prevented by taurine and vitamins E and C administration in rat striatum in vivo. Biochim Biophys Acta 2007 0.76
205 Evidence that the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome induce oxidative stress in brain of young rats. Int J Dev Neurosci 2009 0.76
206 Neurochemical evidence that pristanic acid impairs energy production and inhibits synaptic Na(+), K(+)-ATPase activity in brain of young rats. Neurochem Res 2011 0.76
207 Erythrocyte glutathione peroxidase activity and plasma selenium concentration are reduced in maple syrup urine disease patients during treatment. Int J Dev Neurosci 2007 0.76
208 Evidence that intracellular Ca2+ mediates the effect of alpha-ketoisocaproic acid on the phosphorylating system of cytoskeletal proteins from cerebral cortex of immature rats. J Neurol Sci 2005 0.76
209 Evidence that 3-hydroxyisobutyric acid inhibits key enzymes of energy metabolism in cerebral cortex of young rats. Int J Dev Neurosci 2008 0.75
210 Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease. Cell Mol Neurobiol 2006 0.75
211 Effect of propionic and methylmalonic acids on the in vitro phosphorylation of intermediate filaments from cerebral cortex of rats during development. Metab Brain Dis 2003 0.75
212 Evidence that antioxidants prevent the inhibition of Na+,K(+)-ATPase activity induced by octanoic acid in rat cerebral cortex in vitro. Neurochem Res 2003 0.75
213 Reduction of glutamate uptake into cerebral cortex of developing rats by the branched-chain alpha-keto acids accumulating in maple syrup urine disease. Neurochem Res 2004 0.75
214 Guanidinoacetate inhibits glutamate uptake in rat striatum of rats at different ages. Neurochem Res 2007 0.75
215 Pristanic acid provokes lipid, protein, and DNA oxidative damage and reduces the antioxidant defenses in cerebellum of young rats. Cerebellum 2014 0.75
216 cis-4-decenoic acid provokes mitochondrial bioenergetic dysfunction in rat brain. Life Sci 2010 0.75
217 Protective effect of L-carnitine on Phenylalanine-induced DNA damage. Metab Brain Dis 2015 0.75
218 Branched-chain amino acids accumulating in maple syrup urine disease induce morphological alterations in C6 glioma cells probably through reactive species. Int J Dev Neurosci 2007 0.75
219 Effect of proline on creatine kinase activity in rat brain. Metab Brain Dis 2003 0.75
220 Brain Na+,K(+)-ATPase inhibition induced by arginine administration is prevented by vitamins E and C. Neurochem Res 2003 0.75
221 Intrastriatal hypoxanthine administration affects Na+,K+-ATPase, acetylcholinesterase and catalase activities in striatum, hippocampus and cerebral cortex of rats. Int J Dev Neurosci 2006 0.75
222 Evidence that 3-hydroxy-3-methylglutaric and 3-methylglutaric acids induce DNA damage in rat striatum. Metab Brain Dis 2015 0.75
223 Tryptophan reduces creatine kinase activity in the brain cortex of rats. Int J Dev Neurosci 2004 0.75
224 Proline reduces creatine kinase activity in the brain cortex of rats. Neurochem Res 2003 0.75
225 Inhibition of creatine kinase activity by cystine in the kidney of young rats. Pediatr Res 2006 0.75
226 Reduction of energy metabolism in rat hippocampus by arginine administration. Brain Res 2003 0.75
227 Arginine administration reduces catalase activity in midbrain of rats. Neuroreport 2002 0.75
228 Inhibition of creatine kinase activity from rat cerebral cortex by 3-hydroxykynurenine. Brain Res 2006 0.75
229 Evaluation of the mechanism underlying the inhibitory effect of guanidinoacetate on brain Na+, K+-ATPase activity. Int J Dev Neurosci 2004 0.75
230 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
231 Alpha-ketoisocaproic acid increases phosphorylation of intermediate filament proteins from rat cerebral cortex by mechanisms involving Ca2+ and cAMP. Neurochem Res 2005 0.75
232 NMDA Receptors and Oxidative Stress Induced by the Major Metabolites Accumulating in HMG Lyase Deficiency Mediate Hypophosphorylation of Cytoskeletal Proteins in Brain From Adolescent Rats: Potential Mechanisms Contributing to the Neuropathology of This Disease. Neurotox Res 2015 0.75
233 Ornithine In Vivo Administration Disrupts Redox Homeostasis and Decreases Synaptic Na(+), K (+)-ATPase Activity in Cerebellum of Adolescent Rats: Implications for the Pathogenesis of Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH) Syndrome. Cell Mol Neurobiol 2015 0.75
234 Influence of ketone bodies on oxidative stress parameters in brain of developing rats in vitro. Metab Brain Dis 2008 0.75
235 Cysteamine prevents and reverses the inhibition of pyruvate kinase activity caused by cystine in rat heart. Biochim Biophys Acta 2004 0.75
236 Inhibition of the mitochondrial respiratory chain by alanine in rat cerebral cortex. Metab Brain Dis 2002 0.75
237 Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex. Neurochem Int 2005 0.75
238 Protective effect of antioxidants on brain oxidative damage caused by proline administration. Neurosci Res 2005 0.75
239 Reduction of butyrylcholinesterase activity in plasma from patients with disorders of propionate metabolism is prevented by treatment with L-carnitine and protein restriction. Clin Biochem 2011 0.75
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