| 1 | Evidence That Long‐Term Treatment Prevents Tissue Oxidative Damage in Patients With Inherited Disorders of the Propionate Pathway | 1.7 | 0 | Citations (PDF) |
| 2 | Protective effects of the PPAR agonist bezafibrate against disruption of redox and energy homeostasis, neuronal death, astroglial reactivity, and neuroinflammation induced in vivo by D-2-hydroxyglutaric acid in rat brain | 4.4 | 0 | Citations (PDF) |
| 3 | The Role of Excitotoxicity, Oxidative Stress and Bioenergetics Disruption in the Neuropathology of Nonketotic Hyperglycinemia | 3.0 | 0 | Citations (PDF) |
| 4 | Cerebral White Matter Alterations Associated With Oligodendrocyte Vulnerability in Organic Acidurias: Insights in Glutaric Aciduria Type I | 3.0 | 0 | Citations (PDF) |
| 5 | 3-Hydroxy-3-Methylglutaric Acid Disrupts Brain Bioenergetics, Redox Homeostasis, and Mitochondrial Dynamics and Affects Neurodevelopment in Neonatal Wistar Rats | 3.6 | 1 | Citations (PDF) |
| 6 | Oxidative Stress Associated With Increased Reactive Nitrogen Species Generation in the Liver and Kidney Caused by a Major Metabolite Accumulating in Tyrosinemia Type 1 | 2.7 | 0 | Citations (PDF) |
| 7 | Disruption of mitochondrial bioenergetics, calcium retention capacity and cell viability caused by D-2-hydroxyglutaric acid in the heart | 3.0 | 2 | Citations (PDF) |
| 8 | Sulfite Impairs Bioenergetics and Redox Status in Neonatal Rat Brain: Insights into the Early Neuropathophysiology of Isolated Sulfite Oxidase and Molybdenum Cofactor Deficiencies | 4.1 | 7 | Citations (PDF) |
| 9 | N-Acetylglutamate and N-acetylmethionine compromise mitochondrial bioenergetics homeostasis and glutamate oxidation in brain of developing rats: Potential implications for the pathogenesis of ACY1 deficiency | 2.1 | 1 | Citations (PDF) |
| 10 | Disturbance of mitochondrial functions caused by N-acetylglutamate and N-acetylmethionine in brain of adolescent rats: Potential relevance in aminoacylase 1 deficiency | 3.6 | 0 | Citations (PDF) |
| 11 | In Vivo Intracerebral Administration of α-Ketoisocaproic Acid to Neonate Rats Disrupts Brain Redox Homeostasis and Promotes Neuronal Death, Glial Reactivity, and Myelination Injury | 3.8 | 3 | Citations (PDF) |
| 12 | Increased susceptibility to quinolinic acid‐induced seizures and long‐term changes in brain oscillations in an animal model of glutaric acidemia type I | 3.3 | 0 | Citations (PDF) |
| 13 | Plinia trunciflora Extract Administration Prevents HI-Induced Oxidative Stress, Inflammatory Response, Behavioral Impairments, and Tissue Damage in Rats | 4.6 | 4 | Citations (PDF) |
| 14 | Antioxidant system disturbances and mitochondrial dysfunction induced by 3-methyglutaric acid in rat heart are prevented by bezafibrate | 4.4 | 9 | Citations (PDF) |
| 15 | Pathophysiology of maple syrup urine disease: Focus on the neurotoxic role of the accumulated branched-chain amino acids and branched-chain α-keto acids | 3.6 | 21 | Citations (PDF) |
| 16 | Disturbance of Mitochondrial Dynamics, Endoplasmic Reticulum–Mitochondria Crosstalk, Redox Homeostasis, and Inflammatory Response in the Brain of Glutaryl-CoA Dehydrogenase-Deficient Mice: Neuroprotective Effects of Bezafibrate | 3.8 | 10 | Citations (PDF) |
| 17 | l-2-Hydroxyglutaric Acid Administration to Neonatal Rats Elicits Marked Neurochemical Alterations and Long-Term Neurobehavioral Disabilities Mediated by Oxidative Stress | 3.0 | 3 | Citations (PDF) |
| 18 | The mitochondrial‐targeted reactive species scavenger
JP4
‐039 prevents sulfite‐induced alterations in antioxidant defenses, energy transfer, and cell death signaling in striatum of rats | 3.4 | 8 | Citations (PDF) |
| 19 | 3-Hydroxyglutaric Acid as a Neurotoxin 2021, , 1-20 | | 0 | Citations (PDF) |
| 20 | Glutaric Acid Neurotoxicity: Mechanisms and Actions 2021, , 1-35 | | 3 | Citations (PDF) |
| 21 | Ethylmalonic acid impairs bioenergetics by disturbing succinate and glutamate oxidation and induces mitochondrial permeability transition pore opening in rat cerebellum | 4.0 | 5 | Citations (PDF) |
| 22 | L-carnitine protects DNA oxidative damage induced by phenylalanine and its keto acid derivatives in neural cells: a possible pathomechanism and adjuvant therapy for brain injury in phenylketonuria | 3.0 | 7 | Citations (PDF) |
| 23 | S-adenosylmethionine induces mitochondrial dysfunction, permeability transition pore opening and redox imbalance in subcellular preparations of rat liver | 2.7 | 3 | Citations (PDF) |
| 24 | Protective effects of L-carnitine on behavioral alterations and neuroinflammation in striatum of glutaryl-COA dehydrogenase deficient mice | 2.7 | 9 | Citations (PDF) |
| 25 | Neuronal Death, Glial Reactivity, Microglia Activation, Oxidative Stress and Bioenergetics Impairment Caused by Intracerebroventricular Administration of D-2-hydroxyglutaric Acid to Neonatal Rats | 2.5 | 10 | Citations (PDF) |
| 26 | Glutaric Acidemia Type 1: An Inherited Neurometabolic Disorder of Intoxication 2021, , 1-25 | | 1 | Citations (PDF) |
| 27 | Impairment of mitochondrial bioenergetics and permeability transition induction caused by major long-chain fatty acids accumulating in VLCAD deficiency in skeletal muscle as potential pathomechanisms of myopathy | 2.7 | 10 | Citations (PDF) |
| 28 | Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats | 4.1 | 11 | Citations (PDF) |
| 29 | Elevated levels of BDNF and cathepsin‐<scp>d</scp>as possible peripheral markers of neurodegeneration in plasma of patients with glutaric acidemia type I | 1.6 | 9 | Citations (PDF) |
| 30 | Oxidative damage in mitochondrial fatty acids oxidation disorders patients and the in vitro effect of l-carnitine on DNA damage induced by the accumulated metabolites | 2.7 | 20 | Citations (PDF) |
| 31 | Disruption of mitochondrial functions and oxidative stress contribute to neurologic dysfunction in organic acidurias | 2.7 | 11 | Citations (PDF) |
| 32 | Mitochondrial Dysfunction and Redox Homeostasis Impairment as Pathomechanisms of Brain Damage in Ethylmalonic Encephalopathy: Insights from Animal and Human Studies | 4.1 | 13 | Citations (PDF) |
| 33 | Lipopolysaccharide-Elicited Systemic Inflammation Induces Selective Vulnerability of Cerebral Cortex and Striatum of Developing Glutaryl-CoA Dehydrogenase Deficient (Gcdh−/−) Mice to Oxidative Stress | 3.0 | 8 | Citations (PDF) |
| 34 | Recent Advances in the Pathophysiology of Fatty Acid Oxidation Defects: Secondary Alterations of Bioenergetics and Mitochondrial Calcium Homeostasis Caused by the Accumulating Fatty Acids | 2.4 | 12 | Citations (PDF) |
| 35 | In vivo evidence that bezafibrate prevents oxidative stress and mitochondrial dysfunction caused by 3-methylglutaric acid in rat liver | 3.0 | 12 | Citations (PDF) |
| 36 | Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats | 4.1 | 13 | Citations (PDF) |
| 37 | Acute Liver Failure Induces Glial Reactivity, Oxidative Stress and Impairs Brain Energy Metabolism in Rats | 3.5 | 7 | Citations (PDF) |
| 38 | Clinical, biochemical and molecular findings of 24 Brazilian patients with glutaric acidemia type 1: 4 novel mutations in the GCDH gene | 3.0 | 8 | Citations (PDF) |
| 39 | Monitoring of Phenylalanine Levels in Patients with Phenylketonuria Using Dried Blood Spots: a Comparison of Two Methods | 0.2 | 3 | Citations (PDF) |
| 40 | Anandamide Reduces the Toxic Synergism Exerted by Quinolinic Acid and Glutaric Acid in Rat Brain Neuronal Cells | 2.5 | 12 | Citations (PDF) |
| 41 | Acute lysine overload provokes marked striatum injury involving oxidative stress signaling pathways in glutaryl-CoA dehydrogenase deficient mice | 3.6 | 10 | Citations (PDF) |
| 42 | Pathogenesis of brain damage in glutaric acidemia type I: Lessons from the genetic mice model | 1.6 | 19 | Citations (PDF) |
| 43 | l-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload | 4.1 | 11 | Citations (PDF) |
| 44 | Prevention by L-carnitine of DNA damage induced by 3-hydroxy-3-methylglutaric and 3-methylglutaric acids and experimental evidence of lipid and DNA damage in patients with 3-hydroxy-3-methylglutaric aciduria | 2.7 | 9 | Citations (PDF) |
| 45 | Experimental evidence that maleic acid markedly compromises glutamate oxidation through inhibition of glutamate dehydrogenase and α-ketoglutarate dehydrogenase activities in kidney of developing rats | 3.2 | 8 | Citations (PDF) |
| 46 | Screening for organic acidurias and aminoacidopathies in high-risk Brazilian patients: Eleven-year experience of a reference center | 1.3 | 13 | Citations (PDF) |
| 47 | Neurological manifestations of organic acidurias | 9.2 | 51 | Citations (PDF) |
| 48 | Bezafibrate In Vivo Administration Prevents 3-Methylglutaric Acid-Induced Impairment of Redox Status, Mitochondrial Biogenesis, and Neural Injury in Brain of Developing Rats | 3.0 | 16 | Citations (PDF) |
| 49 | Evidence that thiol group modification and reactive oxygen species are involved in hydrogen sulfide-induced mitochondrial permeability transition pore opening in rat cerebellum | 4.1 | 9 | Citations (PDF) |
| 50 | Free Radical Scavengers Prevent Argininosuccinic Acid-Induced Oxidative Stress in the Brain of Developing Rats: a New Adjuvant Therapy for Argininosuccinate Lyase Deficiency? | 3.8 | 12 | Citations (PDF) |
| 51 | 3-Hydroxy-3-Methylglutaric Acid Impairs Redox and Energy Homeostasis, Mitochondrial Dynamics, and Endoplasmic Reticulum–Mitochondria Crosstalk in Rat Brain | 3.0 | 12 | Citations (PDF) |
| 52 | Metabolite accumulation in <scp>VLCAD</scp> deficiency markedly disrupts mitochondrial bioenergetics and Ca<sup>2+</sup> homeostasis in the heart | 5.5 | 20 | Citations (PDF) |
| 53 | Experimental Evidence that In Vivo Intracerebral Administration of L-2-Hydroxyglutaric Acid to Neonatal Rats Provokes Disruption of Redox Status and Histopathological Abnormalities in the Brain | 3.0 | 19 | Citations (PDF) |
| 54 | The Role of Hyperhomocysteinemia in Disease | 1.3 | 1 | Citations (PDF) |
| 55 | Evidence that Thiosulfate Inhibits Creatine Kinase Activity in Rat Striatum via Thiol Group Oxidation | 3.0 | 20 | Citations (PDF) |
| 56 | Selective Screening of Fatty Acids Oxidation Defects and Organic Acidemias by Liquid Chromatography/tandem Mass Spectrometry Acylcarnitine Analysis in Brazilian Patients | 2.7 | 10 | Citations (PDF) |
| 57 | Oxidative damage in glutaric aciduria type I patients and the protective effects of <scp>l</scp>‐carnitine treatment | 3.1 | 34 | Citations (PDF) |
| 58 | High vulnerability of the heart and liver to 3‐hydroxypalmitic acid–induced disruption of mitochondrial functions in intact cell systems | 3.1 | 3 | Citations (PDF) |
| 59 | Disruption of Brain Redox Homeostasis, Microglia Activation and Neuronal Damage Induced by Intracerebroventricular Administration of S-Adenosylmethionine to Developing Rats | 3.8 | 17 | Citations (PDF) |
| 60 | Long Lasting High Lysine Diet Aggravates White Matter Injury in Glutaryl-CoA Dehydrogenase Deficient (Gcdh−/−) Mice | 3.8 | 11 | Citations (PDF) |
| 61 | The Role of Oxidative Stress and Bioenergetic Dysfunction in Sulfite Oxidase Deficiency: Insights from Animal Models | 3.0 | 29 | Citations (PDF) |
| 62 | Bezafibrate Prevents Glycine-Induced Increase of Antioxidant Enzyme Activities in Rat Striatum | 3.8 | 12 | Citations (PDF) |
| 63 | Oxidative Stress in Homocystinuria Due to Cystathionine ß-Synthase Deficiency: Findings in Patients and in Animal Models | 4.1 | 30 | Citations (PDF) |
| 64 | Experimental evidence of oxidative stress in patients with l-2-hydroxyglutaric aciduria and that l-carnitine attenuates in vitro DNA damage caused by d-2-hydroxyglutaric and l-2-hydroxyglutaric acids | 2.7 | 25 | Citations (PDF) |
| 65 | Disruption of Energy Transfer and Redox Status by Sulfite in Hippocampus, Striatum, and Cerebellum of Developing Rats | 3.0 | 13 | Citations (PDF) |
| 66 | Mechanistic Bases of Neurotoxicity Provoked by Fatty Acids Accumulating in MCAD and LCHAD Deficiencies | 1.3 | 4 | Citations (PDF) |
| 67 | α-Ketoadipic Acid and α-Aminoadipic Acid Cause Disturbance of Glutamatergic Neurotransmission and Induction of Oxidative Stress In Vitro in Brain of Adolescent Rats | 3.0 | 18 | Citations (PDF) |
| 68 | Bioenergetics dysfunction, mitochondrial permeability transition pore opening and lipid peroxidation induced by hydrogen sulfide as relevant pathomechanisms underlying the neurological dysfunction characteristic of ethylmalonic encephalopathy | 4.1 | 18 | Citations (PDF) |
| 69 | Intracerebroventricular injection of glycine alters enzymatic antioxidant defenses in rat striatum: prevention by bezafibrate | 3.0 | 0 | Citations (PDF) |
| 70 | Bezafibrate prevents mitochondrial dysfunction, antioxidant system disturbance, glial reactivity and neuronal damage induced by sulfite administration in striatum of rats: Implications for a possible therapeutic strategy for sulfite oxidase deficiency | 4.1 | 49 | Citations (PDF) |
| 71 | Mevalonolactone disrupts mitochondrial functions and induces permeability transition pore opening in rat brain mitochondria: Implications for the pathogenesis of mevalonic aciduria | 3.6 | 7 | Citations (PDF) |
| 72 | Impairment of <scp>GABA</scp>ergic system contributes to epileptogenesis in glutaric acidemia type I | 4.8 | 13 | Citations (PDF) |
| 73 | Induction of Neuroinflammatory Response and Histopathological Alterations Caused by Quinolinic Acid Administration in the Striatum of Glutaryl-CoA Dehydrogenase Deficient Mice | 3.0 | 6 | Citations (PDF) |
| 74 | Glycine Administration Alters MAPK Signaling Pathways and Causes Neuronal Damage in Rat Brain: Putative Mechanisms Involved in the Neurological Dysfunction in Nonketotic Hyperglycinemia | 3.8 | 10 | Citations (PDF) |
| 75 | S-Adenosylmethionine Promotes Oxidative Stress and Decreases Na+, K+-ATPase Activity in Cerebral Cortex Supernatants of Adolescent Rats: Implications for the Pathogenesis of S-Adenosylhomocysteine Hydrolase Deficiency | 3.8 | 10 | Citations (PDF) |
| 76 | Toxic Synergism Between Quinolinic Acid and Glutaric Acid in Neuronal Cells Is Mediated by Oxidative Stress: Insights to a New Toxic Model | 3.8 | 13 | Citations (PDF) |
| 77 | Chronic Exposure to β-Alanine Generates Oxidative Stress and Alters Energy Metabolism in Cerebral Cortex and Cerebellum of Wistar Rats | 3.8 | 21 | Citations (PDF) |
| 78 | A Possible Anti-Inflammatory Effect of Proline in the Brain Cortex and Cerebellum of Rats | 3.8 | 16 | Citations (PDF) |
| 79 | 2‐Methylcitric acid impairs glutamate metabolism and induces permeability transition in brain mitochondria | 4.0 | 30 | Citations (PDF) |
| 80 | Signaling mechanisms underlying the glioprotective effects of resveratrol against mitochondrial dysfunction | 4.1 | 35 | Citations (PDF) |
| 81 | Mitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studies | 3.9 | 147 | Citations (PDF) |
| 82 | Higher susceptibility of cerebral cortex and striatum to sulfite neurotoxicity in sulfite oxidase-deficient rats | 4.1 | 14 | Citations (PDF) |
| 83 | Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle | 2.7 | 21 | Citations (PDF) |
| 84 | 3-Hydroxy-3-methylglutaric and 3-methylglutaric acids impair redox status and energy production and transfer in rat heart: relevance for the pathophysiology of cardiac dysfunction in 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency | 2.9 | 19 | Citations (PDF) |
| 85 | cis-4-Decenoic and decanoic acids impair mitochondrial energy, redox and Ca 2+ homeostasis and induce mitochondrial permeability transition pore opening in rat brain and liver: Possible implications for the pathogenesis of MCAD deficiency | 0.6 | 16 | Citations (PDF) |
| 86 | Ornithine and Homocitrulline Impair Mitochondrial Function, Decrease Antioxidant Defenses and Induce Cell Death in Menadione-Stressed Rat Cortical Astrocytes: Potential Mechanisms of Neurological Dysfunction in HHH Syndrome | 3.5 | 18 | Citations (PDF) |
| 87 | Experimental Evidence that 3-Methylglutaric Acid Disturbs Mitochondrial Function and Induced Oxidative Stress in Rat Brain Synaptosomes: New Converging Mechanisms | 3.5 | 14 | Citations (PDF) |
| 88 | Evaluation of Oxidative Stress Parameters and Energy Metabolism in Cerebral Cortex of Rats Subjected to Sarcosine Administration | 3.8 | 5 | Citations (PDF) |
| 89 | Higher Vulnerability of Menadione-Exposed Cortical Astrocytes of Glutaryl-CoA Dehydrogenase Deficient Mice to Oxidative Stress, Mitochondrial Dysfunction, and Cell Death: Implications for the Neurodegeneration in Glutaric Aciduria Type I | 3.8 | 7 | Citations (PDF) |
| 90 | Deregulation of mitochondrial functions provoked by long‐chain fatty acid accumulating in long‐chain 3‐hydroxyacyl‐CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart – mitochondrial permeability transition pore opening as a potential contributing pathomechanism of cardiac alterations in these disorders | 5.5 | 19 | Citations (PDF) |
| 91 | Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation | 3.0 | 32 | Citations (PDF) |
| 92 | Striatal neuronal death mediated by astrocytes from the Gcdh−/− mouse model of glutaric acidemia type I | 3.1 | 26 | Citations (PDF) |
| 93 | Experimental evidence that bioenergetics disruption is not mainly involved in the brain injury of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload | 2.5 | 13 | Citations (PDF) |
| 94 | Disturbance of redox homeostasis as a contributing underlying pathomechanism of brain and liver alterations in 3‐hydroxy‐3‐methylglutaryl‐CoA lyase deficiency | 3.4 | 18 | Citations (PDF) |
| 95 | Disturbance of energy and redox homeostasis and reduction of Na+,K+-ATPase activity provoked by in vivo intracerebral administration of ethylmalonic acid to young rats | 4.1 | 16 | Citations (PDF) |
| 96 | Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria | 3.2 | 25 | Citations (PDF) |
| 97 | Protective effect of L-carnitine on Phenylalanine-induced DNA damage | 3.0 | 16 | Citations (PDF) |
| 98 | Urinary biomarkers of oxidative damage in Maple syrup urine disease: The <scp>l</scp>‐carnitine role | 1.6 | 29 | Citations (PDF) |
| 99 | <i>In vitro</i> evidence that sulfite impairs glutamatergic neurotransmission and inhibits glutathione metabolism‐related enzymes in rat cerebral cortex | 1.6 | 18 | Citations (PDF) |
| 100 | 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 | 3.0 | 7 | Citations (PDF) |
| 101 | Reactive nitrogen species mediate oxidative stress and astrogliosis provoked by in vivo administration of phytanic acid in cerebellum of adolescent rats: A potential contributing pathomechanism of cerebellar injury in peroxisomal disorders | 2.5 | 20 | Citations (PDF) |
| 102 | Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency | 0.6 | 18 | Citations (PDF) |
| 103 | In vivo intracerebral administration of L-2-hydroxyglutaric acid provokes oxidative stress and histopathological alterations in striatum and cerebellum of adolescent rats | 3.0 | 24 | Citations (PDF) |
| 104 | l-Carnitine supplementation decreases DNA damage in treated MSUD patients | 1.8 | 30 | Citations (PDF) |
| 105 | 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 | 4.1 | 4 | Citations (PDF) |
| 106 | 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 | 4.1 | 19 | Citations (PDF) |
| 107 | Evidence that 3-hydroxy-3-methylglutaric and 3-methylglutaric acids induce DNA damage in rat striatum | 3.0 | 8 | Citations (PDF) |
| 108 | Urinary biomarkers of oxidative stress and plasmatic inflammatory profile in phenylketonuric treated patients | 1.6 | 45 | Citations (PDF) |
| 109 | Toxic synergism between quinolinic acid and organic acids accumulating in glutaric acidemia type I and in disorders of propionate metabolism in rat brain synaptosomes: Relevance for metabolic acidemias | 2.5 | 22 | Citations (PDF) |
| 110 | The effect of WIN 55,212-2 suggests a cannabinoid-sensitive component in the early toxicity induced by organic acids accumulating in glutaric acidemia type I and in related disorders of propionate metabolism in rat brain synaptosomes | 2.5 | 13 | Citations (PDF) |
| 111 | Experimental evidence that overexpression of NR2B glutamate receptor subunit is associated with brain vacuolation in adult glutaryl-CoA dehydrogenase deficient mice: A potential role for glutamatergic-induced excitotoxicity in GA I neuropathology | 1.4 | 15 | Citations (PDF) |
| 112 | Cannabinoid receptor agonists reduce the short-term mitochondrial dysfunction and oxidative stress linked to excitotoxicity in the rat brain | 2.5 | 47 | Citations (PDF) |
| 113 | Oxidative Stress, Disrupted Energy Metabolism, and Altered Signaling Pathways in Glutaryl-CoA Dehydrogenase Knockout Mice: Potential Implications of Quinolinic Acid Toxicity in the Neuropathology of Glutaric Acidemia Type I | 3.8 | 35 | Citations (PDF) |
| 114 | Induction of a Proinflammatory Response in Cortical Astrocytes by the Major Metabolites Accumulating in HMG-CoA Lyase Deficiency: the Role of ERK Signaling Pathway in Cytokine Release | 3.8 | 14 | Citations (PDF) |
| 115 | Intracerebral Glycine Administration Impairs Energy and Redox Homeostasis and Induces Glial Reactivity in Cerebral Cortex of Newborn Rats | 3.8 | 20 | Citations (PDF) |
| 116 | Oxidative Stress in Patients with X-Linked Adrenoleukodystrophy | 4.1 | 21 | Citations (PDF) |
| 117 | Pristanic Acid Provokes Lipid, Protein, and DNA Oxidative Damage and Reduces the Antioxidant Defenses in Cerebellum of Young Rats | 2.1 | 8 | Citations (PDF) |
| 118 | Homocysteine contribution to DNA damage in cystathionine β-synthase-deficient patients | 2.4 | 16 | Citations (PDF) |
| 119 | Ethylmalonic Acid Induces Permeability Transition in Isolated Brain Mitochondria | 3.0 | 11 | Citations (PDF) |
| 120 | Biochemical, histopathological and behavioral alterations caused by intrastriatal administration of quinolic acid to young rats | 5.5 | 23 | Citations (PDF) |
| 121 | Sulfite disrupts brain mitochondrial energy homeostasis and induces mitochondrial permeability transition pore opening via thiol group modification | 4.1 | 35 | Citations (PDF) |
| 122 | l-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders | 2.4 | 185 | Citations (PDF) |
| 123 | Disturbance of the glutamatergic system by glutaric acid in striatum and cerebral cortex of glutaryl-CoA dehydrogenase-deficient knockout mice: Possible implications for the neuropathology of glutaric acidemia type I | 1.4 | 13 | Citations (PDF) |
| 124 | Acute lysine overload provokes protein oxidative damage and reduction of antioxidant defenses in the brain of infant glutaryl-CoA dehydrogenase deficient mice: A role for oxidative stress in GA I neuropathology | 1.4 | 13 | Citations (PDF) |
| 125 | Evidence that glycine induces lipid peroxidation and decreases glutathione concentrations in rat cerebellum | 3.2 | 11 | Citations (PDF) |
| 126 | Disruption of oxidative phosphorylation and synaptic Na+, K+-ATPase activity by pristanic acid in cerebellum of young rats | 4.5 | 0 | Citations (PDF) |
| 127 | Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: A possible role of mPTP opening as a pathomechanism in these disorders? | 4.1 | 25 | Citations (PDF) |
| 128 | Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium‐chain acyl‐CoA dehydrogenase deficiency | 3.4 | 18 | Citations (PDF) |
| 129 | Increased Glutamate Receptor and Transporter Expression in the Cerebral Cortex and Striatum of Gcdh-/- Mice: Possible Implications for the Neuropathology of Glutaric Acidemia Type I | 2.5 | 21 | Citations (PDF) |
| 130 | Glycine Intracerebroventricular Administration Disrupts Mitochondrial Energy Homeostasis in Cerebral Cortex and Striatum of Young Rats | 3.0 | 13 | Citations (PDF) |
| 131 | Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice | 4.5 | 10 | Citations (PDF) |
| 132 | Disturbance of brain energy and redox homeostasis provoked by sulfite and thiosulfate: Potential pathomechanisms involved in the neuropathology of sulfite oxidase deficiency | 2.4 | 36 | Citations (PDF) |
| 133 | Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation | 0.9 | 29 | Citations (PDF) |
| 134 | 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 | 0.9 | 22 | Citations (PDF) |
| 135 | Disturbance of redox homeostasis by ornithine and homocitrulline in rat cerebellum: A possible mechanism of cerebellar dysfunction in HHH syndrome | 4.5 | 18 | Citations (PDF) |
| 136 | Protein and lipid damage in maple syrup urine disease patients: <scp>l</scp>‐carnitine effect | 1.6 | 42 | Citations (PDF) |
| 137 | Resveratrol Protects C6 Astrocyte Cell Line against Hydrogen Peroxide-Induced Oxidative Stress through Heme Oxygenase 1 | 2.5 | 116 | Citations (PDF) |
| 138 | Neurological Damage in MSUD: The Role of Oxidative Stress | 4.1 | 49 | Citations (PDF) |
| 139 | Chronic postnatal ornithine administration to rats provokes learning deficit in the open field task | 3.0 | 4 | Citations (PDF) |
| 140 | Impairment of brain redox homeostasis caused by the major metabolites accumulating in hyperornithinemia–hyperammonemia–homocitrullinuria syndrome in vivo | 3.0 | 9 | Citations (PDF) |
| 141 | Reduction of butyrylcholinesterase activity in plasma from patients with disorders of propionate metabolism is prevented by treatment with L-carnitine and protein restriction | 1.8 | 3 | Citations (PDF) |
| 142 | Marked reduction of Na+, K+-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice | 0.9 | 24 | Citations (PDF) |
| 143 | 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 | 0.9 | 24 | Citations (PDF) |
| 144 | Ethylmalonic acid impairs brain mitochondrial succinate and malate transport | 0.9 | 16 | Citations (PDF) |
| 145 | Induction of oxidative stress in brain of glutaryl-CoA dehydrogenase deficient mice by acute lysine administration | 0.9 | 28 | Citations (PDF) |
| 146 | Pyruvate Kinase Activity and δ-Aminolevulinic Acid Dehydratase Activity as Biomarkers of Toxicity in Workers Exposed to Lead | 2.2 | 23 | Citations (PDF) |
| 147 | Disruption of redox homeostasis in cerebral cortex of developing rats by acylcarnitines accumulating in medium‐chain acyl‐CoA dehydrogenase deficiency | 1.6 | 15 | Citations (PDF) |
| 148 | Oxidative stress in Niemann‐Pick type C patients: a protective role of N‐butyl‐deoxynojirimycin therapy | 1.6 | 39 | Citations (PDF) |
| 149 | 2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain | 2.5 | 12 | Citations (PDF) |
| 150 | Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease | 3.2 | 13 | Citations (PDF) |
| 151 | Signaling mechanisms downstream of quinolinic acid targeting the cytoskeleton of rat striatal neurons and astrocytes | 4.1 | 36 | Citations (PDF) |
| 152 | Experimental evidence that pristanic acid disrupts mitochondrial homeostasis in brain of young rats | 3.3 | 8 | Citations (PDF) |
| 153 | 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 | 2.7 | 37 | Citations (PDF) |
| 154 | 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 | 2.7 | 14 | Citations (PDF) |
| 155 | Neurochemical Evidence that the Metabolites Accumulating in 3-Methylcrotonyl-CoA Carboxylase Deficiency Induce Oxidative Damage in Cerebral Cortex of Young Rats | 4.1 | 14 | Citations (PDF) |
| 156 | Disruption of Mitochondrial Homeostasis by Phytanic Acid in Cerebellum of Young Rats | 2.1 | 16 | Citations (PDF) |
| 157 | Neurochemical evidence that 3‐methylglutaric acid inhibits synaptic Na<sup>+</sup>,K<sup>+</sup>‐ATPase activity probably through oxidative damage in brain cortex of young rats | 1.6 | 28 | Citations (PDF) |
| 158 | Glycine intrastriatal administration induces lipid and protein oxidative damage and alters the enzymatic antioxidant defenses in rat brain | 4.5 | 13 | Citations (PDF) |
| 159 | Experimental evidence of oxidative stress in plasma of homocystinuric patients: A possible role for homocysteine | 0.9 | 38 | Citations (PDF) |
| 160 | Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia–hyperammonemia–homocitrullinuria syndrome | 2.5 | 15 | Citations (PDF) |
| 161 | Pristanic acid promotes oxidative stress in brain cortex of young rats: A possible pathophysiological mechanism for brain damage in peroxisomal disorders | 2.5 | 15 | Citations (PDF) |
| 162 | Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studies | 2.7 | 66 | Citations (PDF) |
| 163 | Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia | 2.7 | 59 | Citations (PDF) |
| 164 | Experimental Evidence that Methylmalonic Acid Provokes Oxidative Damage and Compromises Antioxidant Defenses in Nerve Terminal and Striatum of Young Rats | 4.1 | 50 | Citations (PDF) |
| 165 | Oxidative Stress in Phenylketonuria: What is the Evidence? | 4.1 | 66 | Citations (PDF) |
| 166 | Neurochemical Evidence that Pristanic Acid Impairs Energy Production and Inhibits Synaptic Na+, K+-ATPase Activity in Brain of Young Rats | 3.5 | 10 | Citations (PDF) |
| 167 | Oxidative Stress Parameters in Urine from Patients with Disorders of Propionate Metabolism: a Beneficial Effect of l-Carnitine Supplementation | 4.1 | 31 | Citations (PDF) |
| 168 | 3-Methylcrotonylglycine Disrupts Mitochondrial Energy Homeostasis and Inhibits Synaptic Na+,K+-ATPase Activity in Brain of Young Rats | 4.1 | 15 | Citations (PDF) |
| 169 | Toxicity of octanoate and decanoate in rat peripheral tissues: evidence of bioenergetic dysfunction and oxidative damage induction in liver and skeletal muscle | 3.2 | 27 | Citations (PDF) |
| 170 | The molecular landscape of propionic acidemia and methylmalonic aciduria in Latin America | 3.4 | 15 | Citations (PDF) |
| 171 | Acute administration of 5-oxoproline induces oxidative damage to lipids and proteins and impairs antioxidant defenses in cerebral cortex and cerebellum of young rats | 3.0 | 35 | Citations (PDF) |
| 172 | Proline impairs energy metabolism in cerebral cortex of young rats | 3.0 | 21 | Citations (PDF) |
| 173 | N-acetylaspartic acid impairs enzymatic antioxidant defenses and enhances hydrogen peroxide concentration in rat brain | 3.0 | 5 | Citations (PDF) |
| 174 | Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I | 3.0 | 14 | Citations (PDF) |
| 175 | Evidence that 2-methylacetoacetate induces oxidative stress in rat brain | 3.0 | 7 | Citations (PDF) |
| 176 | α-Ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain | 2.5 | 77 | Citations (PDF) |
| 177 | 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 | 2.5 | 24 | Citations (PDF) |
| 178 | An overview of L-2-hydroxyglutarate dehydrogenase gene (L2HGDH) variants: a genotype-phenotype study | 4.1 | 100 | Citations (PDF) |
| 179 | Simvastatin treatment prevents oxidative damage to DNA in whole blood leukocytes of dyslipidemic type 2 diabetic patients | 2.7 | 29 | Citations (PDF) |
| 180 | Lipoic acid prevents oxidative stress in vitro and in vivo by an acute hyperphenylalaninemia chemically-induced in rat brain | 1.4 | 40 | Citations (PDF) |
| 181 | Prevention by l-carnitine of DNA damage induced by propionic and l-methylmalonic acids in human peripheral leukocytes in vitro | 2.1 | 49 | Citations (PDF) |
| 182 | Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brain | 3.6 | 31 | Citations (PDF) |
| 183 | Neurochemical evidence that glycine induces bioenergetical dysfunction | 3.6 | 23 | Citations (PDF) |
| 184 | Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of <scp>l</scp>‐carnitine supplementation | 1.6 | 49 | Citations (PDF) |
| 185 | <scp>d</scp>‐Serine administration provokes lipid oxidation and decreases the antioxidant defenses in rat striatum | 1.6 | 9 | Citations (PDF) |
| 186 | Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain | 4.5 | 30 | Citations (PDF) |
| 187 | cis-4-decenoic acid provokes mitochondrial bioenergetic dysfunction in rat brain | 4.5 | 11 | Citations (PDF) |
| 188 | Neurochemical evidence that phytanic acid induces oxidative damage and reduces the antioxidant defenses in cerebellum and cerebral cortex of rats | 4.5 | 33 | Citations (PDF) |
| 189 | Acute intrastriatal administration of quinolinic acid provokes hyperphosphorylation of cytoskeletal intermediate filament proteins in astrocytes and neurons of rats | 4.1 | 64 | Citations (PDF) |
| 190 | Neurochemical Evidence that Lysine Inhibits Synaptic Na+,K+-ATPase Activity and Provokes Oxidative Damage in Striatum of Young Rats In vivo | 3.5 | 11 | Citations (PDF) |
| 191 | D-Serine induces lipid and protein oxidative damage and decreases glutathione levels in brain cortex of rats | 2.5 | 12 | Citations (PDF) |
| 192 | Creatine administration prevents Na+,K+-ATPase inhibition induced by intracerebroventricular administration of isovaleric acid in cerebral cortex of young rats | 2.5 | 9 | Citations (PDF) |
| 193 | Experimental evidence that ornithine and homocitrulline disrupt energy metabolism in brain of young rats | 2.5 | 18 | Citations (PDF) |
| 194 | Evidence that the major metabolites accumulating in medium-chain acyl-CoA dehydrogenase deficiency disturb mitochondrial energy homeostasis in rat brain | 2.5 | 31 | Citations (PDF) |
| 195 | In vitro evidence that d-serine disturbs the citric acid cycle through inhibition of citrate synthase activity in rat cerebral cortex | 2.5 | 8 | Citations (PDF) |
| 196 | Inhibition of creatine kinase activity by lysine in rat cerebral cortex | 3.0 | 11 | Citations (PDF) |
| 197 | Effects of 1,4-butanediol administration on oxidative stress in rat brain: Study of the neurotoxicity of γ-hydroxybutyric acid in vivo | 3.0 | 31 | Citations (PDF) |
| 198 | Intracerebroventricular administration of N-acetylaspartic acid impairs antioxidant defenses and promotes protein oxidation in cerebral cortex of rats | 3.0 | 21 | Citations (PDF) |
| 199 | Evidence that folic acid deficiency is a major determinant of hyperhomocysteinemia in Parkinson´s disease | 3.0 | 60 | Citations (PDF) |
| 200 | Tyrosine administration decreases glutathione and stimulates lipid and protein oxidation in rat cerebral cortex | 3.0 | 31 | Citations (PDF) |
| 201 | Amino acids levels and lipid peroxidation in maple syrup urine disease patients | 1.8 | 24 | Citations (PDF) |
| 202 | Selective screening for organic acidemias by urine organic acid GC–MS analysis in Brazil: Fifteen-year experience | 1.2 | 43 | Citations (PDF) |
| 203 | Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit | 2.3 | 10 | Citations (PDF) |
| 204 | Effect of short‐ and long‐term exposition to high phenylalanine blood levels on oxidative damage in phenylketonuric patients | 1.6 | 53 | Citations (PDF) |
| 205 | Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3‐hydroxy‐3‐methylglutaryl‐CoA lyase deficiency as compared to liver | 1.6 | 21 | Citations (PDF) |
| 206 | Evidence that the major metabolites accumulating in hyperornithinemia–hyperammonemia–homocitrullinuria syndrome induce oxidative stress in brain of young rats | 1.6 | 9 | Citations (PDF) |
| 207 | Evidence that DNA damage is associated to phenylalanine blood levels in leukocytes from phenylketonuric patients | 2.1 | 39 | Citations (PDF) |
| 208 | Medium-chain fatty acids accumulating in MCAD deficiency elicit lipid and protein oxidative damage and decrease non-enzymatic antioxidant defenses in rat brain | 3.6 | 30 | Citations (PDF) |
| 209 | Experimental Evidence that Phenylalanine Provokes Oxidative Stress in Hippocampus and Cerebral Cortex of Developing Rats | 4.1 | 57 | Citations (PDF) |
| 210 | Promotion of Lipid and Protein Oxidative Damage in Rat Brain by Ethylmalonic Acid | 3.5 | 16 | Citations (PDF) |
| 211 | Tryptophan administration induces oxidative stress in brain cortex of rats | 3.0 | 20 | Citations (PDF) |
| 212 | Influence of ketone bodies on oxidative stress parameters in brain of developing rats in vitro | 3.0 | 10 | Citations (PDF) |
| 213 | Guanidinoacetate Decreases Antioxidant Defenses and Total Protein Sulfhydryl Content in Striatum of Rats | 3.5 | 46 | Citations (PDF) |
| 214 | Evidence that 3‐hydroxy‐3‐methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex | 3.3 | 29 | Citations (PDF) |
| 215 | Maple syrup urine disease in treated patients: Biochemical and oxidative stress profiles | 1.8 | 24 | Citations (PDF) |
| 216 | Astrocytic proliferation and mitochondrial dysfunction induced by accumulated glutaric acidemia I (GAI) metabolites: Possible implications for GAI pathogenesis | 5.2 | 43 | Citations (PDF) |
| 217 | Oxidative stress is induced in female carriers of X-linked adrenoleukodystrophy | 1.4 | 18 | Citations (PDF) |
| 218 | S12.32 Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial dicarboxylate transport: Implications for the methylmalonic acidemia physiophatology | 0.6 | 0 | Citations (PDF) |
| 219 | Evidence that 3‐hydroxyisobutyric acid inhibits key enzymes of energy metabolism in cerebral cortex of young rats | 1.6 | 12 | Citations (PDF) |
| 220 | Tyrosine promotes oxidative stress in cerebral cortex of young rats | 1.6 | 36 | Citations (PDF) |
| 221 | Lysine induces lipid and protein damage and decreases reduced glutathione concentrations in brain of young rats | 1.6 | 21 | Citations (PDF) |
| 222 | Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats | 4.5 | 33 | Citations (PDF) |
| 223 | Chronic early postnatal glutaric acid administration causes cognitive deficits in the water maze | 2.3 | 9 | Citations (PDF) |
| 224 | Induction of oxidative stress by the metabolites accumulating in isovaleric acidemia in brain cortex of young rats | 2.9 | 22 | Citations (PDF) |
| 225 | l-Carnitine Blood Levels and Oxidative Stress in Treated Phenylketonuric Patients | 4.1 | 58 | Citations (PDF) |
| 226 | Glycine Provokes Lipid Oxidative Damage and Reduces the Antioxidant Defenses in Brain Cortex of Young Rats | 4.1 | 25 | Citations (PDF) |
| 227 | 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 | 4.1 | 18 | Citations (PDF) |
| 228 | Evidence that glutaric acid reduces glutamate uptake by cerebral cortex of infant rats | 4.5 | 31 | Citations (PDF) |
| 229 | In vitro evidence for an antioxidant role of 3-hydroxykynurenine and 3-hydroxyanthranilic acid in the brain | 3.6 | 77 | Citations (PDF) |
| 230 | γ-Hydroxybutyric acid induces oxidative stress in cerebral cortex of young rats | 3.6 | 40 | Citations (PDF) |
| 231 | In vitro effect of quinolinic acid on energy metabolism in brain of young rats | 2.2 | 24 | Citations (PDF) |
| 232 | Branched‐chain amino acids accumulating in maple syrup urine disease induce morphological alterations in C6 glioma cells probably through reactive species | 1.6 | 14 | Citations (PDF) |
| 233 | N
‐Acetylaspartic acid promotes oxidative stress in cerebral cortex of rats | 1.6 | 22 | Citations (PDF) |
| 234 | Erythrocyte glutathione peroxidase activity and plasma selenium concentration are reduced in maple syrup urine disease patients during treatment | 1.6 | 23 | Citations (PDF) |
| 235 | Evidence for a synergistic action of glutaric and 3‐hydroxyglutaric acids disturbing rat brain energy metabolism | 1.6 | 34 | Citations (PDF) |
| 236 | Induction of lipid peroxidation and decrease of antioxidant defenses in symptomatic and asymptomatic patients with X‐linked adrenoleukodystrophy | 1.6 | 32 | Citations (PDF) |
| 237 | Oxidative stress induction by<i>cis</i>-4-decenoic acid: Relevance for MCAD deficiency | 2.9 | 19 | Citations (PDF) |
| 238 | Effect of the branched-chain α-keto acids accumulating in maple syrup urine disease on S100B release from glial cells | 1.4 | 16 | Citations (PDF) |
| 239 | Chemically induced model of hypermethioninemia in rats | 2.4 | 32 | Citations (PDF) |
| 240 | Arginine administration reduces creatine kinase activity in rat cerebellum | 3.0 | 10 | Citations (PDF) |
| 241 | 5-Oxoproline Reduces Non-Enzymatic Antioxidant Defenses in vitro in Rat Brain | 3.0 | 35 | Citations (PDF) |
| 242 | Energy Metabolism is Compromised in Skeletal Muscle of Rats Chronically-Treated with Glutaric Acid | 3.0 | 12 | Citations (PDF) |
| 243 | Synaptic Plasma Membrane Na+, K+-ATPase Activity is Significantly Reduced by the α-Keto Acids Accumulating in Maple Syrup Urine Disease in Rat Cerebral Cortex | 3.0 | 8 | Citations (PDF) |
| 244 | Guanidinoacetate Inhibits Glutamate Uptake in Rat Striatum of Rats at Different Ages | 3.5 | 16 | Citations (PDF) |
| 245 | Induction of Oxidative Stress by Chronic and Acute Glutaric Acid Administration to Rats | 4.1 | 51 | Citations (PDF) |
| 246 | Isovaleric Acid Reduces Na+, K+-ATPase Activity in Synaptic Membranes from Cerebral Cortex of Young Rats | 4.1 | 36 | Citations (PDF) |
| 247 | Age and Brain Structural Related Effects of Glutaric and 3-Hydroxyglutaric Acids on Glutamate Binding to Plasma Membranes During Rat Brain Development | 4.1 | 21 | Citations (PDF) |
| 248 | Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester | 1.9 | 14 | Citations (PDF) |
| 249 | Oxidative stress in plasma from maple syrup urine disease patients during treatment | 3.0 | 38 | Citations (PDF) |
| 250 | Effects of cysteamine on oxidative status in cerebral cortex of rats | 3.0 | 14 | Citations (PDF) |
| 251 | Hexacosanoic and docosanoic acids plasma levels in patients with cerebral childhood and asymptomatic X-linked adrenoleukodystrophy: Lorenzo’s oil effect | 3.0 | 19 | Citations (PDF) |
| 252 | Inhibition of Brain Energy Metabolism by the Branched-chain Amino Acids Accumulating in Maple Syrup Urine Disease | 3.5 | 55 | Citations (PDF) |
| 253 | Antioxidant Effect of Cysteamine in Brain Cortex of Young Rats | 3.5 | 39 | Citations (PDF) |
| 254 | Inhibition of Creatine Kinase Activity by Cystine in the Kidney of Young Rats | 2.1 | 4 | Citations (PDF) |
| 255 | The effect of Lorenzo's oil on oxidative stress in X-linked adrenoleukodystrophy | 1.4 | 21 | Citations (PDF) |
| 256 | Promotion of oxidative stress by l-tryptophan in cerebral cortex of rats | 3.6 | 28 | Citations (PDF) |
| 257 | Morphological alterations and induction of oxidative stress in glial cells caused by the branched-chain α-keto acids accumulating in maple syrup urine disease | 3.6 | 48 | Citations (PDF) |
| 258 | Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues | 1.6 | 44 | Citations (PDF) |
| 259 | Folic acid pretreatment prevents the reduction of Na
+
,K
+
‐ATPase and butyrylcholinesterase activities in rats subjected to acute hyperhomocysteinemia | 1.6 | 19 | Citations (PDF) |
| 260 | Intrastriatal hypoxanthine administration affects Na
+
,K
+
‐ATPase, acetylcholinesterase and catalase activities in striatum, hippocampus and cerebral cortex of rats | 1.6 | 7 | Citations (PDF) |
| 261 | Evidence that quinolinic acid severely impairs energy metabolism through activation of NMDA receptors in striatum from developing rats | 4.0 | 54 | Citations (PDF) |
| 262 | A chemically-induced acute model of maple syrup urine disease in rats for neurochemical studies | 2.4 | 30 | Citations (PDF) |
| 263 | α-Tocopherol and Ascorbic Acid Administration Prevents the Impairment of Brain Energy Metabolism of Hyperargininemic Rats | 4.1 | 17 | Citations (PDF) |
| 264 | The Human Serotonin 1A Receptor Expressed in Neuronal Cells: Toward a Native Environment for Neuronal Receptors | 4.1 | 16 | Citations (PDF) |
| 265 | Inhibition of the Electron Transport Chain and Creatine Kinase Activity by Ethylmalonic Acid in Human Skeletal Muscle | 3.0 | 24 | Citations (PDF) |
| 266 | Propionic Acid Induces Cytoskeletal Alterations in Cultured Astrocytes From Rat Cerebral Cortex | 3.0 | 24 | Citations (PDF) |
| 267 | Intrastriatal Administration of Guanidinoacetate Inhibits Na+, K+-ATPase and Creatine Kinase Activities in Rat Striatum | 3.0 | 36 | Citations (PDF) |
| 268 | Effect of In Vivo Administration of Ethylmalonic Acid on Energy Metabolism in Rat Tissues | 3.0 | 12 | Citations (PDF) |
| 269 | Citrulline and Ammonia Accumulating in Citrullinemia Reduces Antioxidant Capacity of Rat Brain In Vitro | 3.0 | 11 | Citations (PDF) |
| 270 | Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease | 3.0 | 74 | Citations (PDF) |
| 271 | Inhibition of creatine kinase activity from rat cerebral cortex by 3-hydroxykynurenine | 2.5 | 5 | Citations (PDF) |
| 272 | Natural History, Outcome, and Treatment Efficacy in Children and Adults with Glutaryl-CoA Dehydrogenase Deficiency | 2.1 | 224 | Citations (PDF) |
| 273 | Kynurenines Impair Energy Metabolism in Rat Cerebral Cortex | 4.1 | 26 | Citations (PDF) |
| 274 | Effect of hypoxanthine on Na+,K+-ATPase activity and some parameters of oxidative stress in rat striatum | 2.5 | 16 | Citations (PDF) |
| 275 | Reduction of Butyrylcholinesterase Activity in Rat Serum Subjected to Hyperhomocysteinemia | 3.0 | 28 | Citations (PDF) |
| 276 | α-Keto Acids Accumulating in Maple Syrup Urine Disease Stimulate Lipid Peroxidation and Reduce Antioxidant Defences in Cerebral Cortex From Young Rats | 3.0 | 69 | Citations (PDF) |
| 277 | Branched-Chain α-Keto Acids Accumulating in Maple Syrup Urine Disease Induce Reorganization of Phosphorylated GFAP in C6-Glioma Cells | 3.0 | 20 | Citations (PDF) |
| 278 | Glutaric Acid Administration Impairs Energy Metabolism in Midbrain and Skeletal Muscle of Young Rats | 3.5 | 30 | Citations (PDF) |
| 279 | Morphological Alterations and Cell Death Provoked by the Branched-Chain α-Amino Acids Accumulating in Maple Syrup Urine Disease in Astrocytes from Rat Cerebral Cortex | 4.1 | 15 | Citations (PDF) |
| 280 | Alpha-Ketoisocaproic Acid Increases Phosphorylation of Intermediate Filament Proteins from Rat Cerebral Cortex by Mechanisms Involving Ca2+ and cAMP | 3.5 | 13 | Citations (PDF) |
| 281 | Evaluation of the mechanisms involved in leucine-induced oxidative damage in cerebral cortex of young rats | 2.9 | 51 | Citations (PDF) |
| 282 | Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex | 3.6 | 16 | Citations (PDF) |
| 283 | Protective effect of antioxidants on brain oxidative damage caused by proline administration | 2.2 | 17 | Citations (PDF) |
| 284 | The effects of the interactions between amino acids on pyruvate kinase activity from the brain cortex of young rats | 1.6 | 16 | Citations (PDF) |
| 285 | Methionine alters Na
+
,K
+
‐ATPase activity, lipid peroxidation and nonenzymatic antioxidant defenses in rat hippocampus | 1.6 | 45 | Citations (PDF) |
| 286 | Glutaric acid moderately compromises energy metabolism in rat brain | 1.6 | 25 | Citations (PDF) |
| 287 | Quinolinic acid reduces the antioxidant defenses in cerebral cortex of young rats | 1.6 | 44 | Citations (PDF) |
| 288 | Propionic and methylmalonic acids increase cAMP levels in slices of cerebral cortex of young rats via adrenergic and glutamatergic mechanisms | 4.1 | 6 | Citations (PDF) |
| 289 | Mitochondrial energy metabolism is markedly impaired by d-2-hydroxyglutaric acid in rat tissues | 0.9 | 85 | Citations (PDF) |
| 290 | Intrahippocampal administration of the branched-chain α-hydroxy acids accumulating in maple syrup urine disease compromises rat performance in aversive and non-aversive behavioral tasks | 1.4 | 13 | Citations (PDF) |
| 291 | Evidence that intracellular Ca2+ mediates the effect of α-ketoisocaproic acid on the phosphorylating system of cytoskeletal proteins from cerebral cortex of immature rats | 1.4 | 13 | Citations (PDF) |
| 292 | Mitochondrial permeability transition in neuronal damage promoted by Ca2+ and respiratory chain complex II inhibition | 4.0 | 76 | Citations (PDF) |
| 293 | Intrahippocampal administration of the α-keto acids accumulating in maple syrup urine disease provokes learning deficits in rats | 2.4 | 8 | Citations (PDF) |
| 294 | Inhibition of pyruvate kinase activity by cystine in brain cortex of rats | 2.5 | 18 | Citations (PDF) |
| 295 | Inhibition of energy metabolism in cerebral cortex of young rats by the medium-chain fatty acids accumulating in MCAD deficiency | 2.5 | 32 | Citations (PDF) |
| 296 | Arginine Administration Decreases Cerebral Cortex Acetylcholinesterase and Serum Butyrylcholinesterase Probably by Oxidative Stress Induction | 3.5 | 38 | Citations (PDF) |
| 297 | Reduction of Glutamate Uptake into Cerebral Cortex of Developing Rats by the Branched-Chain Alpha-Keto Acids Accumulating in Maple Syrup Urine Disease | 3.5 | 18 | Citations (PDF) |
| 298 | Evidence that the branched-chain ?-keto acids accumulating in maple syrup urine disease induce morphological alterations and death in cultured astrocytes from rat cerebral cortex | 5.2 | 28 | Citations (PDF) |
| 299 | α-Keto-β-methylvaleric acid increases the in vitro phosphorylation of intermediate filaments in cerebral cortex of young rats through the gabaergic system | 1.4 | 11 | Citations (PDF) |
| 300 | In vitro effects of d-2-hydroxyglutaric acid on glutamate binding, uptake and release in cerebral cortex of rats | 1.4 | 20 | Citations (PDF) |
| 301 | Cysteamine prevents and reverses the inhibition of pyruvate kinase activity caused by cystine in rat heart | 4.1 | 5 | Citations (PDF) |
| 302 | Evidence that 3-hydroxyglutaric acid interacts with NMDA receptors in synaptic plasma membranes from cerebral cortex of young rats | 3.6 | 42 | Citations (PDF) |
| 303 | Glutaric acid stimulates glutamate binding and astrocytic uptake and inhibits vesicular glutamate uptake in forebrain from young rats | 3.6 | 33 | Citations (PDF) |
| 304 | Inhibition of Na
+
, K
+
‐ATPase activity in rat striatum by the metabolites accumulated in Lesch–Nyhan disease | 1.6 | 24 | Citations (PDF) |
| 305 | Tryptophan reduces creatine kinase activity in the brain cortex of rats | 1.6 | 11 | Citations (PDF) |
| 306 | Evaluation of the mechanism underlying the inhibitory effect of guanidinoacetate on brain Na
+
, K
+
‐ATPase activity | 1.6 | 20 | Citations (PDF) |
| 307 | Inhibition of creatine kinase activity from rat cerebral cortex by -2-hydroxyglutaric acid in vitro | 3.6 | 45 | Citations (PDF) |
| 308 | Title is missing! | 3.5 | 23 | Citations (PDF) |
| 309 | Title is missing! | 3.0 | 24 | Citations (PDF) |
| 310 | Title is missing! | 3.0 | 81 | Citations (PDF) |
| 311 | Title is missing! | 3.0 | 1 | Citations (PDF) |
| 312 | Title is missing! | 3.5 | 20 | Citations (PDF) |
| 313 | Title is missing! | 3.5 | 17 | Citations (PDF) |
| 314 | Title is missing! | 3.5 | 5 | Citations (PDF) |
| 315 | Title is missing! | 3.0 | 12 | Citations (PDF) |
| 316 | Title is missing! | 3.0 | 23 | Citations (PDF) |
| 317 | Title is missing! | 3.0 | 16 | Citations (PDF) |
| 318 | In Vitro Homocysteine Inhibits Platelet Na+, K+-ATPase and Serum Butyrylcholinesterase Activities of Young Rats | 3.0 | 5 | Citations (PDF) |
| 319 | Title is missing! | 3.0 | 37 | Citations (PDF) |
| 320 | Title is missing! | 3.0 | 23 | Citations (PDF) |
| 321 | Title is missing! | 3.0 | 15 | Citations (PDF) |
| 322 | Title is missing! | 3.5 | 28 | Citations (PDF) |
| 323 | Title is missing! | 3.5 | 36 | Citations (PDF) |
| 324 | In vivo and in vitro effects of proline on some parameters of oxidative stress in rat brain | 2.5 | 33 | Citations (PDF) |
| 325 | Glutaric acid induces oxidative stress in brain of young rats | 2.5 | 81 | Citations (PDF) |
| 326 | Characterization of the inhibition of pyruvate kinase caused by phenylalanine and phenylpyruvate in rat brain cortex | 2.5 | 35 | Citations (PDF) |
| 327 | Lower in vivo brain extracellular GABA concentration in diabetic rats during forced swimming | 2.5 | 29 | Citations (PDF) |
| 328 | Ascorbic acid prevents water maze behavioral deficits caused by early postnatal methylmalonic acid administration in the rat | 2.5 | 30 | Citations (PDF) |
| 329 | Reduction of energy metabolism in rat hippocampus by arginine administration | 2.5 | 24 | Citations (PDF) |
| 330 | Induction of oxidative stress by L-2-hydroxyglutaric acid in rat brain | 3.3 | 54 | Citations (PDF) |
| 331 | D-2-hydroxyglutaric acid induces oxidative stress in cerebral cortex of young rats | 3.7 | 95 | Citations (PDF) |
| 332 | Evaluation of the effect of chronic administration of drugs on rat behavior in the water maze task | 2.4 | 9 | Citations (PDF) |
| 333 | Inhibition of brain energy metabolism by the α-keto acids accumulating in maple syrup urine disease | 4.1 | 78 | Citations (PDF) |
| 334 | Brain energy metabolism is compromised by the metabolites accumulating in homocystinuria | 3.6 | 43 | Citations (PDF) |
| 335 | Hyperphenylalaninemia reduces creatine kinase activity in the cerebral cortex of rats | 1.6 | 27 | Citations (PDF) |
| 336 | Proline induces oxidative stress in cerebral cortex of rats | 1.6 | 54 | Citations (PDF) |
| 337 | Kinetic studies on the inhibition of creatine kinase activity by branched‐chain α‐amino acids in the brain cortex of rats | 1.6 | 10 | Citations (PDF) |
| 338 | l
‐2‐Hydroxyglutaric acid inhibits mitochondrial creatine kinase activity from cerebellum of developing rats | 1.6 | 30 | Citations (PDF) |
| 339 | Inhibition of Na
+
, K
+
‐ATPase activity in rat striatum by guanidinoacetate | 1.6 | 20 | Citations (PDF) |
| 340 | Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease | 1.6 | 74 | Citations (PDF) |
| 341 | Evidence that oxidative stress is involved in the inhibitory effect of proline on Na
+
,K
+
‐ATPase activity in synaptic plasma membrane of rat hippocampus | 1.6 | 29 | Citations (PDF) |
| 342 | Impairment of energy metabolism in hippocampus of rats subjected to chemically-induced hyperhomocysteinemia | 4.1 | 37 | Citations (PDF) |
| 343 | In vitro phosphorylation of cytoskeletal proteins from cerebral cortex of rats | 2.4 | 41 | Citations (PDF) |
| 344 | Arginine administration reduces catalase activity in midbrain of rats | 1.5 | 14 | Citations (PDF) |
| 345 | Inhibition of cytochrome c oxidase activity in rat cerebral cortex and human skeletal muscle by d-2-hydroxyglutaric acid in vitro | 4.1 | 80 | Citations (PDF) |
| 346 | Experimental hyperphenylalaninemia provokes oxidative stress in rat brain | 4.1 | 56 | Citations (PDF) |
| 347 | On the mechanism of the inhibition of Na
+
, K
+
‐ATPase activity caused by homocysteine | 1.6 | 18 | Citations (PDF) |
| 348 | Ascorbic acid prevents cognitive deficits caused by chronic administration of propionic acid to rats in the water maze | 2.4 | 56 | Citations (PDF) |
| 349 | α-Ketoisocaproic acid regulates phosphorylation of intermediate filaments in postnatal rat cortical slices through ionotropic glutamatergic receptors | 2.2 | 29 | Citations (PDF) |
| 350 | Detection of Organic Acidemias in Brazil | 2.7 | 10 | Citations (PDF) |
| 351 | 3-hydroxyglutaric acid induces oxidative stress and decreases the antioxidant defenses in cerebral cortex of young rats | 2.5 | 63 | Citations (PDF) |
| 352 | Chronic treatment with glutaric acid induces partial tolerance to excitotoxicity in neuronal cultures from chick embryo telencephalons | 3.3 | 53 | Citations (PDF) |
| 353 | Title is missing! | 3.0 | 63 | Citations (PDF) |
| 354 | Title is missing! | 3.5 | 35 | Citations (PDF) |
| 355 | Title is missing! | 3.0 | 49 | Citations (PDF) |
| 356 | Title is missing! | 3.0 | 4 | Citations (PDF) |
| 357 | Title is missing! | 3.5 | 15 | Citations (PDF) |
| 358 | Title is missing! | 4.1 | 110 | Citations (PDF) |
| 359 | Title is missing! | 3.5 | 94 | Citations (PDF) |
| 360 | Title is missing! | 3.5 | 84 | Citations (PDF) |
| 361 | Title is missing! | 3.5 | 40 | Citations (PDF) |
| 362 | Inhibition of Glutamate Uptake into Synaptic Vesicles from Rat Brain by 3-Nitropropionic Acid in Vitro | 4.1 | 20 | Citations (PDF) |
| 363 | Title is missing! | 3.5 | 41 | Citations (PDF) |
| 364 | Title is missing! | 3.5 | 45 | Citations (PDF) |
| 365 | Pharmacological evidence that α-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats | 2.5 | 21 | Citations (PDF) |
| 366 | Intrastriatal administration of 3-hydroxyglutaric acid induces convulsions and striatal lesions in rats | 2.5 | 41 | Citations (PDF) |
| 367 | Effects of methylmalonic and propionic acids on glutamate uptake by synaptosomes and synaptic vesicles and on glutamate release by synaptosomes from cerebral cortex of rats | 2.5 | 22 | Citations (PDF) |
| 368 | In vitro stimulation of oxidative stress in cerebral cortex of rats by the guanidino compounds accumulating in hyperargininemia | 2.5 | 30 | Citations (PDF) |
| 369 | Reduced Na+,K+-ATPase Activity in Erythrocyte Membranes from Patients with Phenylketonuria | 2.1 | 9 | Citations (PDF) |
| 370 | Methylmalonate administration decreases Na+,K+-ATPase activity in cerebral cortex of rats | 1.5 | 118 | Citations (PDF) |
| 371 | Propionic and L-methylmalonic acids induce oxidative stress in brain of young rats | 1.5 | 82 | Citations (PDF) |
| 372 | Quinolinic acid inhibits glutamate uptake into synaptic vesicles from rat brain | 1.5 | 85 | Citations (PDF) |
| 373 | Alpha-ketoisocaproate increases the in vitro 32P incorporation into intermediate filaments in cerebral cortex of rats | 1.5 | 9 | Citations (PDF) |
| 374 | Methylmalonic and propionic acids increase the in vitro incorporation of into cytoskeletal proteins from cerebral cortex of young rats through NMDA glutamate receptors | 2.5 | 25 | Citations (PDF) |
| 375 | Platelet Na+,K+-ATPase activity as a possible peripheral marker for the neurotoxic effects of phenylalanine in phenylketonuria | 3.0 | 3 | Citations (PDF) |
| 376 | Clinical and biochemical findings in 7 patients with X-linked adrenoleukodystrophy treated with Lorenzo's Oil | 1.3 | 2 | Citations (PDF) |
| 377 | Inhibition of glutamate uptake into synaptic vesicles of rat brain by the metabolites accumulating in maple syrup urine disease | 1.4 | 59 | Citations (PDF) |
| 378 | Inhibition of synaptosomal [3H]glutamate uptake and [3H]glutamate binding to plasma membranes from brain of young rats by glutaric acid in vitro | 1.4 | 52 | Citations (PDF) |
| 379 | Title is missing! | 3.0 | 23 | Citations (PDF) |
| 380 | Title is missing! | 3.0 | 18 | Citations (PDF) |
| 381 | In vitro inhibition of Na+,K+-ATPase activity from rat cerebral cortex by guanidino compounds accumulating in hyperargininemia | 2.5 | 38 | Citations (PDF) |
| 382 | Effect of Chemically Induced Propionic Acidemia on Neurobehavioral Development of Rats | 2.4 | 55 | Citations (PDF) |
| 383 | Inhibition of mitogen-activated proliferation of human peripheral lymphocytes in vitro by propionic acid | 6.3 | 13 | Citations (PDF) |
| 384 | Ascorbic acid and α-tocopherol attenuate methylmalonic acid-induced convulsions | 1.5 | 59 | Citations (PDF) |
| 385 | Pharmacological evidence for GABAergic and glutamatergic involvement in the convulsant and behavioral effects of glutaric acid | 2.5 | 47 | Citations (PDF) |
| 386 | Effects of acute and chronic administration of methylmalonic and propionic acids on the in vitro incorporation of 32P into cytoskeletal proteins from cerebral cortex of young rats | 3.6 | 4 | Citations (PDF) |
| 387 | Chronic administration of propionic acid reduces ganglioside N-acetylneuraminic acid concentration in cerebellum of young rats | 1.4 | 15 | Citations (PDF) |
| 388 | Inhibition of Na+,K+-ATPase from rat brain cortex by propionic acid | 1.5 | 49 | Citations (PDF) |
| 389 | In VitroPhosphorylation of Cytoskeletal Proteins in the Rat Cerebral Cortex Is Decreased by Propionic Acid | 4.1 | 12 | Citations (PDF) |
| 390 | Detection of metabolic disorders in high-risk patients: a pilot study in Salvador, Bahia | 1.3 | 2 | Citations (PDF) |
| 391 | Intrastriatal methylmalonic acid administration induces rotational behavior and convulsions through glutamatergic mechanisms | 2.5 | 67 | Citations (PDF) |
| 392 | Alanine reverses the inhibitory effect of phenylalanine and its metabolites on Na+,K+-ATPase in synaptic plasma membranes from cerebral cortex of rats | 4.2 | 18 | Citations (PDF) |
| 393 | Inhibition of citrate oxidation in vitro by 2-hydroxybutyrate and 4-hydroxybutyrate in cerebral cortex of young rats | 4.2 | 3 | Citations (PDF) |
| 394 | Neurochemical effects ofl-pyroglutamic acid | 3.5 | 5 | Citations (PDF) |
| 395 | 2-Hydroxybutyrate and 4-hydroxybutyrate inhibit CO2 formation from labeled substrates by rat cerebral cortex | 4.2 | 13 | Citations (PDF) |
| 396 | Effect of phenylalanine and its metabolites on ATP diphosphohydrolase activity in synaptosomes from rat cerebral cortex | 3.5 | 23 | Citations (PDF) |
| 397 | Immunomodulatory action of amino acids on lymphocyte blastogenesis | 4.2 | 1 | Citations (PDF) |
| 398 | High urinary excretion of N‐(pyrrole‐2‐carboxyl) glycine in type II hyperprolinemia | 2.2 | 7 | Citations (PDF) |
| 399 | Reduced Locomotor Activity of Rats Made Histidinemic by Injection of Histidine | 3.0 | 11 | Citations (PDF) |
| 400 | Inborn Errors of Metabolism | 1.1 | 2 | Citations (PDF) |
| 401 | Effect of proline administration on rat behavior in aversive and nonaversive tasks | 2.4 | 41 | Citations (PDF) |
| 402 | Distribution of xanthine dehydrogenase and oxidase activities in human and rabbit tissues | 2.0 | 87 | Citations (PDF) |
| 403 | Diminished concentrations of ganglioside N-acetylneuraminic acid (G-NeuAc) in cerebellum of young rats receiving chronic administration of methylmalonic acid | 1.4 | 18 | Citations (PDF) |
| 404 | Relationship between serum immunoglobulin G and alpha-fetoprotein levels during human pregnancy | 1.3 | 3 | Citations (PDF) |
| 405 | Relationship between some obstetric landmarks and the concentration of alpha-fetoprotein in maternal blood | 1.3 | 2 | Citations (PDF) |
| 406 | An improved specific laboratory test for homocystinuria | 1.2 | 8 | Citations (PDF) |
