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List of works by Moacir Wajner

2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain ⬇️

scientific article published on August 29, 2012

2-Methylcitric acid impairs glutamate metabolism and induces permeability transition in brain mitochondria

scientific article

3-Hydroxy-3-Methylglutaric Acid Impairs Redox and Energy Homeostasis, Mitochondrial Dynamics, and Endoplasmic Reticulum-Mitochondria Crosstalk in Rat Brain

scientific article published on 13 November 2019

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.

scientific article published on 18 July 2016

3-Hydroxyglutaric acid induces oxidative stress and decreases the antioxidant defenses in cerebral cortex of young rats

scientific article

3-Methylcrotonylglycine disrupts mitochondrial energy homeostasis and inhibits synaptic Na(+),K (+)-ATPase activity in brain of young rats

scientific article published on 13 October 2011

5-Oxoproline reduces non-enzymatic antioxidant defenses in vitro in rat brain.

scientific article

A Possible Anti-Inflammatory Effect of Proline in the Brain Cortex and Cerebellum of Rats.

scientific article

A chemically-induced acute model of maple syrup urine disease in rats for neurochemical studies

scientific article published in September 2006

Acidúrias orgânicas: diagnóstico em pacientes de alto risco no Brasil

Acute Liver Failure Induces Glial Reactivity, Oxidative Stress and Impairs Brain Energy Metabolism in Rats

scientific article published on 01 January 2019

Acute administration of 5-oxoproline induces oxidative damage to lipids and proteins and impairs antioxidant defenses in cerebral cortex and cerebellum of young rats

scientific article published on 30 April 2010

Acute intrastriatal administration of quinolinic acid provokes hyperphosphorylation of cytoskeletal intermediate filament proteins in astrocytes and neurons of rats

scientific article published on 18 March 2010

Acute lysine overload provokes marked striatum injury involving oxidative stress signaling pathways in glutaryl-CoA dehydrogenase deficient mice

scientific article published on 20 May 2019

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.

scientific article published on 22 June 2014

Age and brain structural related effects of glutaric and 3-hydroxyglutaric acids on glutamate binding to plasma membranes during rat brain development

scientific article published on 05 September 2007

Alanine prevents the in vitro inhibition of glycolysis caused by phenylalanine in brain cortex of rats

scientific article published in March 2003

Alanine prevents the inhibition of pyruvate kinase activity caused by tryptophan in cerebral cortex of rats

scientific article

Alanine prevents the reduction of pyruvate kinase activity in brain cortex of rats subjected to chemically induced hyperphenylalaninemia.

scientific article published in September 2002

Alpha-keto-beta-methylvaleric acid increases the in vitro phosphorylation of intermediate filaments in cerebral cortex of young rats through the gabaergic system

scientific article published in January 2004

Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain

scientific article published on 11 February 2010

Alpha-ketoisocaproic acid increases phosphorylation of intermediate filament proteins from rat cerebral cortex by mechanisms involving Ca2+ and cAMP.

scientific article

Alpha-tocopherol and ascorbic acid administration prevents the impairment of brain energy metabolism of hyperargininemic rats.

scientific article

Amino acids levels and lipid peroxidation in maple syrup urine disease patients.

scientific article

An overview of L-2-hydroxyglutarate dehydrogenase gene (L2HGDH) variants: a genotype-phenotype study

scientific article

Anandamide Reduces the Toxic Synergism Exerted by Quinolinic Acid and Glutaric Acid in Rat Brain Neuronal Cells

scientific article published on 19 January 2019

Antioxidant effect of cysteamine in brain cortex of young rats

scientific article published on 17 October 2007

Arginine administration decreases cerebral cortex acetylcholinesterase and serum butyrylcholinesterase probably by oxidative stress induction

scientific journal article

Arginine administration reduces catalase activity in midbrain of rats

scientific article published in July 2002

Arginine administration reduces creatine kinase activity in rat cerebellum

scientific article

Ascorbic acid prevents cognitive deficits caused by chronic administration of propionic acid to rats in the water maze

scientific article published in October 2002

Ascorbic acid prevents water maze behavioral deficits caused by early postnatal methylmalonic acid administration in the rat.

scientific article published in June 2003

Astrocytic proliferation and mitochondrial dysfunction induced by accumulated glutaric acidemia I (GAI) metabolites: possible implications for GAI pathogenesis

scientific article

Bezafibrate In Vivo Administration Prevents 3-Methylglutaric Acid-Induced Impairment of Redox Status, Mitochondrial Biogenesis, and Neural Injury in Brain of Developing Rats

scientific article published on 09 March 2019

Bezafibrate Prevents Glycine-Induced Increase of Antioxidant Enzyme Activities in Rat Striatum

scientific article published on 19 April 2018

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 deficien

scientific article published on 18 May 2017

Biochemical, histopathological and behavioral alterations caused by intrastriatal administration of quinolic acid to young rats

scientific article published on 13 March 2014

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.

scientific article published on 14 June 2017

Brain Na+,K(+)-ATPase inhibition induced by arginine administration is prevented by vitamins E and C.

scientific article published in June 2003

Brain energy metabolism is compromised by the metabolites accumulating in homocystinuria

scientific article

Branched-chain alpha-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells

scientific article published on September 2005

Branched-chain amino acids accumulating in maple syrup urine disease induce morphological alterations in C6 glioma cells probably through reactive species

scientific article published on 21 January 2007

Characterization of the inhibition of pyruvate kinase caused by phenylalanine and phenylpyruvate in rat brain cortex

scientific article published in April 2003

Chemically induced model of hypermethioninemia in rats

scientific article published on 14 September 2006

Chronic Exposure to β-Alanine Generates Oxidative Stress and Alters Energy Metabolism in Cerebral Cortex and Cerebellum of Wistar Rats.

scientific article

Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit.

scientific article

Chronic early postnatal glutaric acid administration causes cognitive deficits in the water maze.

scientific article published on 11 October 2007

Chronic postnatal ornithine administration to rats provokes learning deficit in the open field task ⬇️

scientific article published on June 16, 2012

Chronic treatment with glutaric acid induces partial tolerance to excitotoxicity in neuronal cultures from chick embryo telencephalons

scientific article

Citrulline and ammonia accumulating in citrullinemia reduces antioxidant capacity of rat brain in vitro.

scientific article published in March 2006

Clinical, biochemical and molecular findings of 24 Brazilian patients with glutaric acidemia type 1: 4 novel mutations in the GCDH gene

scientific article published on 16 October 2020

Creatine administration prevents Na+,K+-ATPase inhibition induced by intracerebroventricular administration of isovaleric acid in cerebral cortex of young rats.

scientific article published on 13 January 2009

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

scientific article published in February 2006

Creatine kinase activity from rat brain is inhibited by branched-chain amino acids in vitro.

scientific article published in May 2003

Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex

scientific article published in April 2005

Cysteamine prevents and reverses the inhibition of pyruvate kinase activity caused by cystine in rat heart

scientific article

D-2-hydroxyglutaric acid induces oxidative stress in cerebral cortex of young rats

scientific article

D-serine induces lipid and protein oxidative damage and decreases glutathione levels in brain cortex of rats.

scientific article published on 24 December 2008

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 open

scientific article

Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues

scientific article published in February 2006

Disruption of Brain Redox Homeostasis, Microglia Activation and Neuronal Damage Induced by Intracerebroventricular Administration of S-Adenosylmethionine to Developing Rats

scientific article published on 30 July 2018

Disruption of Energy Transfer and Redox Status by Sulfite in Hippocampus, Striatum, and Cerebellum of Developing Rats.

scientific article published on 17 April 2017

Disruption of Mitochondrial Homeostasis by Phytanic Acid in Cerebellum of Young Rats ⬇️

scientific article published on June 1, 2013

Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation.

scientific article published on 9 November 2012

Disruption of mitochondrial functions and oxidative stress contribute to neurologic dysfunction in organic acidurias

scientific article published on 21 October 2020

Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studies

scientific article published on February 2011

Disruption of oxidative phosphorylation and synaptic Na(+), K(+)-ATPase activity by pristanic acid in cerebellum of young rats.

scientific article

Disruption of redox homeostasis in cerebral cortex of developing rats by acylcarnitines accumulating in medium-chain acyl-CoA dehydrogenase deficiency.

scientific article published on 27 March 2012

Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats

scientific article published on 10 January 2020

Disturbance of brain energy and redox homeostasis provoked by sulfite and thiosulfate: potential pathomechanisms involved in the neuropathology of sulfite oxidase deficiency.

scientific article

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

scientific article published on 10 January 2015

Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain

scientific article published on 20 April 2010

Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats

scientific article published on 23 October 2019

Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle

scientific article published on 28 June 2016

Disturbance of redox homeostasis as a contributing underlying pathomechanism of brain and liver alterations in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.

scientific article published on 04 June 2015

Disturbance of redox homeostasis by ornithine and homocitrulline in rat cerebellum: a possible mechanism of cerebellar dysfunction in HHH syndrome.

scientific article

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.

scientific article published on 16 September 2014

Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia–hyperammonemia–homocitrullinuria syndrome ⬇️

scientific article published on 05 November 2010

Effect of hypoxanthine on Na+,K+-ATPase activity and some parameters of oxidative stress in rat striatum

scientific article

Effect of in vivo administration of ethylmalonic acid on energy metabolism in rat tissues

scientific article published in March 2006

Effect of leucine administration on creatine kinase activity in rat brain.

scientific article published in March 2003

Effect of proline on creatine kinase activity in rat brain

scientific article published in June 2003

Effect of propionic and methylmalonic acids on the in vitro phosphorylation of intermediate filaments from cerebral cortex of rats during development

scientific article published on September 1, 2003

Effect of short- and long-term exposition to high phenylalanine blood levels on oxidative damage in phenylketonuric patients.

scientific article published on 20 January 2009

Effect of the branched-chain alpha-keto acids accumulating in maple syrup urine disease on S100B release from glial cells

scientific article published on 17 May 2007

Effects of 1,4-butanediol administration on oxidative stress in rat brain: study of the neurotoxicity of gamma-hydroxybutyric acid in vivo

scientific article

Effects of L-2-hydroxyglutaric acid on various parameters of the glutamatergic system in cerebral cortex of rats.

scientific article published on September 2003

Effects of cysteamine on oxidative status in cerebral cortex of rats.

scientific article published on 21 November 2007

Elevated levels of BDNF and cathepsin-d as possible peripheral markers of neurodegeneration in plasma of patients with glutaric acidemia type I

scientific article published on 16 January 2020

Energy metabolism is compromised in skeletal muscle of rats chronically-treated with glutaric acid.

scientific article

Erythrocyte glutathione peroxidase activity and plasma selenium concentration are reduced in maple syrup urine disease patients during treatment

scientific article published on 17 May 2007

Ethylmalonic acid impairs brain mitochondrial succinate and malate transport

scientific article published on 19 October 2011

Ethylmalonic acid induces permeability transition in isolated brain mitochondria

scientific article published on 21 February 2014

Ethylmalonic acid inhibits mitochondrial creatine kinase activity from cerebral cortex of young rats in vitro.

scientific article published in May 2003

Evaluation of Oxidative Stress Parameters and Energy Metabolism in Cerebral Cortex of Rats Subjected to Sarcosine Administration

scientific article published on 29 June 2016

Evaluation of the effect of chronic administration of drugs on rat behavior in the water maze task ⬇️

scientific article published on October 1, 2003

Evaluation of the mechanism underlying the inhibitory effect of guanidinoacetate on brain Na+, K+-ATPase activity.

scientific article published in June 2004

Evidence for a synergistic action of glutaric and 3-hydroxyglutaric acids disturbing rat brain energy metabolism

scientific article

Evidence that 2-methylacetoacetate induces oxidative stress in rat brain.

scientific article published on 14 September 2010

Evidence that 3-hydroxy-3-methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex.

scientific article published in February 2008

Evidence that 3-hydroxy-3-methylglutaric and 3-methylglutaric acids induce DNA damage in rat striatum

scientific article

Evidence that 3-hydroxyglutaric acid interacts with NMDA receptors in synaptic plasma membranes from cerebral cortex of young rats.

scientific article published on December 2004

Evidence that 3-hydroxyisobutyric acid inhibits key enzymes of energy metabolism in cerebral cortex of young rats

scientific article published on 7 February 2008

Evidence that DNA damage is associated to phenylalanine blood levels in leukocytes from phenylketonuric patients

scientific article published on 7 August 2009

Evidence that Thiosulfate Inhibits Creatine Kinase Activity in Rat Striatum via Thiol Group Oxidation

scientific article published on 28 July 2018

Evidence that antioxidants prevent the inhibition of Na+,K(+)-ATPase activity induced by octanoic acid in rat cerebral cortex in vitro.

scientific article published in August 2003

Evidence that folic acid deficiency is a major determinant of hyperhomocysteinemia in Parkinson's disease.

scientific article published on 18 March 2009

Evidence that glutaric acid reduces glutamate uptake by cerebral cortex of infant rats.

scientific article published on 6 October 2007

Evidence that glycine induces lipid peroxidation and decreases glutathione concentrations in rat cerebellum.

scientific article published on 18 June 2014

Evidence that intracellular Ca2+ mediates the effect of alpha-ketoisocaproic acid on the phosphorylating system of cytoskeletal proteins from cerebral cortex of immature rats

scientific article published on 18 August 2005

Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease

scientific article published on 8 November 2006

Evidence that oxidative stress is involved in the inhibitory effect of proline on Na(+),K(+)-ATPase activity in synaptic plasma membrane of rat hippocampus.

scientific article published in October 2003

Evidence that quinolinic acid severely impairs energy metabolism through activation of NMDA receptors in striatum from developing rats.

scientific article

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

scientific article published in November 2004

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.

scientific article published on 24 February 2007

Evidence that the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome induce oxidative stress in brain of young rats.

scientific article published on 13 August 2009

Evidence that the major metabolites accumulating in medium-chain acyl-CoA dehydrogenase deficiency disturb mitochondrial energy homeostasis in rat brain

scientific article published on 21 August 2009

Evidence that thiol group modification and reactive oxygen species are involved in hydrogen sulfide-induced mitochondrial permeability transition pore opening in rat cerebellum

scientific article published on 03 November 2018

Experimental Evidence that 3-Methylglutaric Acid Disturbs Mitochondrial Function and Induced Oxidative Stress in Rat Brain Synaptosomes: New Converging Mechanisms.

scientific article published on 9 June 2016

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

scientific article published on 6 February 2018

Experimental Evidence that Methylmalonic Acid Provokes Oxidative Damage and Compromises Antioxidant Defenses in Nerve Terminal and Striatum of Young Rats ⬇️

scientific article published on March 22, 2011

Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium-chain acyl-CoA dehydrogenase deficiency.

scientific article published on 13 March 2014

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

scientific article published on 7 April 2017

Experimental evidence of oxidative stress in plasma of homocystinuric patients: A possible role for homocysteine

scientific article published on 24 June 2011

Experimental evidence that bioenergetics disruption is not mainly involved in the brain injury of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload

scientific article

Experimental evidence that maleic acid markedly compromises glutamate oxidation through inhibition of glutamate dehydrogenase and α-ketoglutarate dehydrogenase activities in kidney of developing rats

scientific article published on 29 April 2019

Experimental evidence that ornithine and homocitrulline disrupt energy metabolism in brain of young rats.

scientific article published on 16 July 2009

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.

scientific article published on 28 October 2015

Experimental evidence that phenylalanine provokes oxidative stress in hippocampus and cerebral cortex of developing rats

scientific article published on 23 September 2009

Experimental evidence that pristanic acid disrupts mitochondrial homeostasis in brain of young rats ⬇️

scientific article published on December 20, 2011

Experimental hyperphenylalaninemia provokes oxidative stress in rat brain.

scientific article

Folic acid pretreatment prevents the reduction of Na(+),K(+)-ATPase and butyrylcholinesterase activities in rats subjected to acute hyperhomocysteinemia

scientific journal article

Free Radical Scavengers Prevent Argininosuccinic Acid-Induced Oxidative Stress in the Brain of Developing Rats: a New Adjuvant Therapy for Argininosuccinate Lyase Deficiency?

scientific article published on 09 November 2019

Gamma-hydroxybutyric acid induces oxidative stress in cerebral cortex of young rats

scientific article

Glutaric acid administration impairs energy metabolism in midbrain and skeletal muscle of young rats.

scientific article

Glutaric acid induces oxidative stress in brain of young rats

scientific article

Glutaric acid moderately compromises energy metabolism in rat brain

scientific article

Glutaric acid stimulates glutamate binding and astrocytic uptake and inhibits vesicular glutamate uptake in forebrain from young rats

scientific article

Glycine Administration Alters MAPK Signaling Pathways and Causes Neuronal Damage in Rat Brain: Putative Mechanisms Involved in the Neurological Dysfunction in Nonketotic Hyperglycinemia.

scientific article published on 3 January 2017

Glycine intracerebroventricular administration disrupts mitochondrial energy homeostasis in cerebral cortex and striatum of young rats.

scientific article published on 3 May 2013

Glycine intrastriatal administration induces lipid and protein oxidative damage and alters the enzymatic antioxidant defenses in rat brain.

scientific article

Glycine provokes lipid oxidative damage and reduces the antioxidant defenses in brain cortex of young rats.

scientific article

Guanidinoacetate decreases antioxidant defenses and total protein sulfhydryl content in striatum of rats.

scientific article

Guanidinoacetate inhibits glutamate uptake in rat striatum of rats at different ages

scientific article published on 2 February 2007

Guanosine enhances glutamate uptake in brain cortical slices at normal and excitotoxic conditions

scientific article published in June 2002

Hexacosanoic and docosanoic acids plasma levels in patients with cerebral childhood and asymptomatic X-linked adrenoleukodystrophy: Lorenzo's oil effect.

scientific article published on 17 November 2007

High vulnerability of the heart and liver to 3-hydroxypalmitic acid-induced disruption of mitochondrial functions in intact cell systems

scientific article published on 20 June 2018

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.

scientific article

Higher susceptibility of cerebral cortex and striatum to sulfite neurotoxicity in sulfite oxidase-deficient rats

scientific article

Homocysteine contribution to DNA damage in cystathionine β-synthase-deficient patients

scientific article

Homocysteine inhibits butyrylcholinesterase activity in rat serum.

scientific article published in September 2003

Hyperphenylalaninemia reduces creatine kinase activity in the cerebral cortex of rats

scientific article

Impairment of GABAergic system contributes to epileptogenesis in glutaric acidemia type I.

scientific article published on August 2017

Impairment of brain redox homeostasis caused by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome in vivo.

scientific article published on 15 July 2012

Impairment of energy metabolism in hippocampus of rats subjected to chemically-induced hyperhomocysteinemia

scientific article published in April 2003

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

scientific article published on 16 October 2019

In vitro effect of homocysteine on some parameters of oxidative stress in rat hippocampus

scientific article

In vitro effect of quinolinic acid on energy metabolism in brain of young rats

scientific article published on 28 November 2006

In vitro effects of D-2-hydroxyglutaric acid on glutamate binding, uptake and release in cerebral cortex of rats.

scientific article published on February 2004

In vitro evidence for an antioxidant role of 3-hydroxykynurenine and 3-hydroxyanthranilic acid in the brain

scientific article published on 7 September 2006

In vitro evidence that D-serine disturbs the citric acid cycle through inhibition of citrate synthase activity in rat cerebral cortex

scientific article published on 3 September 2009

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

scientific article published on 29 July 2010

In vitro evidence that sulfite impairs glutamatergic neurotransmission and inhibits glutathione metabolism-related enzymes in rat cerebral cortex.

scientific article

In vitro homocysteine inhibits platelet Na+,K+-ATPase and serum butyrylcholinesterase activities of young rats.

scientific article

In vitro phosphorylation of cytoskeletal proteins from cerebral cortex of rats ⬇️

scientific article published on May 1, 2003

In vivo and in vitro effects of proline on some parameters of oxidative stress in rat brain

scientific article published in November 2003

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 diso

scientific article published on 6 April 2013

In vivo intracerebral administration of L-2-hydroxyglutaric acid provokes oxidative stress and histopathological alterations in striatum and cerebellum of adolescent rats.

scientific article published on 17 February 2015

Inborn Errors of Metabolism

scientific article published in November 1989

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.

scientific article

Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria

scientific article published on 04 January 2015

Induction of Neuroinflammatory Response and Histopathological Alterations Caused by Quinolinic Acid Administration in the Striatum of Glutaryl-CoA Dehydrogenase Deficient Mice.

scientific article published on 12 December 2017

Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I.

scientific article

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

scientific article published on 23 June 2015

Induction of lipid peroxidation and decrease of antioxidant defenses in symptomatic and asymptomatic patients with X-linked adrenoleukodystrophy.

scientific article published in September 2007

Induction of oxidative stress by L-2-hydroxyglutaric acid in rat brain

scientific article published in October 2003

Induction of oxidative stress by chronic and acute glutaric acid administration to rats

scientific article

Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats.

scientific article published on 11 January 2008

Induction of oxidative stress by the metabolites accumulating in isovaleric acidemia in brain cortex of young rats.

scientific article published in August 2008

Induction of oxidative stress in brain of glutaryl-CoA dehydrogenase deficient mice by acute lysine administration ⬇️

scientific article published on March 13, 2012

Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease.

scientific article published in October 2003

Influence of ketone bodies on oxidative stress parameters in brain of developing rats in vitro.

scientific article

Inhibition of Na(+),K(+)-ATPase activity in hippocampus of rats subjected to acute administration of homocysteine is prevented by vitamins E and C treatment

scientific article

Inhibition of Na+, K+-ATPase activity by the metabolites accumulating in homocystinuria.

scientific article published in June 2002

Inhibition of Na+, K+-ATPase activity in rat striatum by guanidinoacetate.

scientific article

Inhibition of Na+, K+-ATPase activity in rat striatum by the metabolites accumulated in Lesch-Nyhan disease

scientific article published in February 2004

Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease

scientific article published in November 2003

Inhibition of brain energy metabolism by the branched-chain amino acids accumulating in maple syrup urine disease

scientific article published on 08 August 2007

Inhibition of creatine kinase activity by cystine in the kidney of young rats

scientific article published on 01 August 2006

Inhibition of creatine kinase activity by lysine in rat cerebral cortex

scientific article

Inhibition of creatine kinase activity from rat cerebral cortex by 3-hydroxykynurenine

scientific article published on 9 November 2006

Inhibition of creatine kinase activity from rat cerebral cortex by D-2-hydroxyglutaric acid in vitro.

scientific article published in January 2004

Inhibition of creatine kinase activity in vitro by ethylmalonic acid in cerebral cortex of young rats.

scientific article

Inhibition of cytochrome c oxidase activity in rat cerebral cortex and human skeletal muscle by D-2-hydroxyglutaric acid in vitro.

scientific article published in January 2002

Inhibition of energy metabolism in cerebral cortex of young rats by the medium-chain fatty acids accumulating in MCAD deficiency

scientific article published in December 2004

Inhibition of mitochondrial creatine kinase activity by D-2-hydroxyglutaric acid in cerebellum of young rats.

scientific article published in September 2003

Inhibition of pyruvate kinase activity by cystine in brain cortex of rats

scientific article

Inhibition of the electron transport chain and creatine kinase activity by ethylmalonic acid in human skeletal muscle.

scientific article published in March 2006

Inhibition of the mitochondrial respiratory chain by alanine in rat cerebral cortex

scientific article published in September 2002

Inhibition of the mitochondrial respiratory chain by phenylalanine in rat cerebral cortex

scientific article

Intracerebral Glycine Administration Impairs Energy and Redox Homeostasis and Induces Glial Reactivity in Cerebral Cortex of Newborn Rats

scientific article published on 26 October 2015

Intracerebroventricular administration of N-acetylaspartic acid impairs antioxidant defenses and promotes protein oxidation in cerebral cortex of rats.

scientific article published on 18 March 2009

Intrahippocampal administration of the alpha-keto acids accumulating in maple syrup urine disease provokes learning deficits in rats

scientific article

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

scientific article

Intrastriatal administration of guanidinoacetate inhibits Na+, K+-ATPase and creatine kinase activities in rat striatum.

scientific article published in March 2006

Intrastriatal hypoxanthine administration affects Na+,K+-ATPase, acetylcholinesterase and catalase activities in striatum, hippocampus and cerebral cortex of rats

scientific article published on 10 October 2006

Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation

scientific article

In vivo evidence that bezafibrate prevents oxidative stress and mitochondrial dysfunction caused by 3-methylglutaric acid in rat liver

scientific article published on 10 March 2020

Isovaleric acid reduces Na+, K+-ATPase activity in synaptic membranes from cerebral cortex of young rats

scientific article published on 30 March 2007

Kinetic studies on the inhibition of creatine kinase activity by branched-chain alpha-amino acids in the brain cortex of rats.

scientific article published in May 2003

Kynurenines impair energy metabolism in rat cerebral cortex

scientific article published on 7 December 2006

L-2-Hydroxyglutaric aciduria: pattern of MR imaging abnormalities in 56 patients

scientific article published on June 2009

L-2-hydroxyglutaric acid inhibits mitochondrial creatine kinase activity from cerebellum of developing rats.

scientific article

L-Carnitine supplementation decreases DNA damage in treated MSUD patients.

scientific article

L-carnitine blood levels and oxidative stress in treated phenylketonuric patients

scientific article published on 24 September 2008

L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders

scientific article published on 19 October 2013

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

scientific article published on 25 March 2015

Lipoic acid prevents oxidative stress in vitro and in vivo by an acute hyperphenylalaninemia chemically-induced in rat brain

scientific article published on 11 March 2010

Lipopolysaccharide-Elicited Systemic Inflammation Induces Selective Vulnerability of Cerebral Cortex and Striatum of Developing Glutaryl-CoA Dehydrogenase Deficient (Gcdh-/-) Mice to Oxidative Stress

scientific article published on 01 October 2020

Long Lasting High Lysine Diet Aggravates White Matter Injury in Glutaryl-CoA Dehydrogenase Deficient (Gcdh-/-) Mice

scientific article published on 19 May 2018

Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brain

scientific article published on 8 April 2010

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

scientific article published on 13 October 2012

Lower in vivo brain extracellular GABA concentration in diabetic rats during forced swimming

scientific article published on April 2003

Lysine induces lipid and protein damage and decreases reduced glutathione concentrations in brain of young rats.

scientific article

Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles.

scientific article

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.

scientific article

Marked reduction of Na(+), K(+)-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice.

scientific article

Medium-chain fatty acids accumulating in MCAD deficiency elicit lipid and protein oxidative damage and decrease non-enzymatic antioxidant defenses in rat brain

scientific article published on 24 February 2009

Metabolite accumulation in VLCAD deficiency markedly disrupts mitochondrial bioenergetics and Ca2+ homeostasis in the heart

scientific article published on 24 February 2018

Methionine alters Na+,K+-ATPase activity, lipid peroxidation and nonenzymatic antioxidant defenses in rat hippocampus.

scientific article published on 10 August 2005

Mevalonolactone disrupts mitochondrial functions and induces permeability transition pore opening in rat brain mitochondria: Implications for the pathogenesis of mevalonic aciduria

scientific article published on 8 March 2017

Mitochondrial Dysfunction and Redox Homeostasis Impairment as Pathomechanisms of Brain Damage in Ethylmalonic Encephalopathy: Insights from Animal and Human Studies

scientific article published on 09 October 2020

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?

scientific article published on 16 June 2014

Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia

scientific article published on February 2011

Mitochondrial energy metabolism is markedly impaired by D-2-hydroxyglutaric acid in rat tissues.

scientific article published on 15 June 2005

Mitochondrial permeability transition in neuronal damage promoted by Ca2+ and respiratory chain complex II inhibition

scientific article

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

scientific article published in August 2005

Morphological alterations and induction of oxidative stress in glial cells caused by the branched-chain alpha-keto acids accumulating in maple syrup urine disease

scientific article published on 5 July 2006

N-acetylaspartic acid impairs enzymatic antioxidant defenses and enhances hydrogen peroxide concentration in rat brain.

scientific article published in May 2010

N-acetylaspartic acid promotes oxidative stress in cerebral cortex of rats.

scientific article published on 7 May 2007

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

scientific article published on 15 July 2015

Natural history, outcome, and treatment efficacy in children and adults with glutaryl-CoA dehydrogenase deficiency

scientific article (publication date: June 2006)

Neurochemical Evidence that Lysine Inhibits Synaptic Na+,K+-ATPase Activity and Provokes Oxidative Damage in Striatum of Young Rats In vivo ⬇️

scientific article published on October 27, 2010

Neurochemical Evidence that Pristanic Acid Impairs Energy Production and Inhibits Synaptic Na+, K+-ATPase Activity in Brain of Young Rats ⬇️

scientific article published on March 29, 2011

Neurochemical evidence that 3-methylglutaric acid inhibits synaptic Na+,K+-ATPase activity probably through oxidative damage in brain cortex of young rats

scientific article published on 02 November 2010

Neurochemical evidence that glycine induces bioenergetical dysfunction

scientific article published on 13 April 2010

Neurochemical evidence that phytanic acid induces oxidative damage and reduces the antioxidant defenses in cerebellum and cerebral cortex of rats.

scientific article published on 6 July 2010

Neurochemical evidence that the metabolites accumulating in 3-methylcrotonyl-CoA carboxylase deficiency induce oxidative damage in cerebral cortex of young rats.

scientific article published on 28 September 2012

Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice ⬇️

scientific article published on November 28, 2012

Neurological damage in MSUD: the role of oxidative stress.

scientific article

Neurological manifestations of organic acidurias.

scientific article published in May 2019

On the mechanism of the inhibition of Na(+), K(+)-ATPase activity caused by homocysteine.

scientific article published in April 2002

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.

scientific article published on 13 March 2015

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

scientific article published on 9 May 2016

Oxidative Stress Parameters in Urine from Patients with Disorders of Propionate Metabolism: a Beneficial Effect of l-Carnitine Supplementation ⬇️

scientific article published on July 22, 2011

Oxidative Stress in Homocystinuria Due to Cystathionine ß-Synthase Deficiency: Findings in Patients and in Animal Models.

scientific article published on 3 March 2017

Oxidative Stress in Patients with X-Linked Adrenoleukodystrophy

scientific article published on 14 July 2015

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.

scientific article published on 25 November 2015

Oxidative damage in glutaric aciduria type I patients and the protective effects of l-carnitine treatment

scientific article published on 20 August 2018

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

scientific article published on 22 November 2019

Oxidative stress in Niemann‐Pick type C patients: a protective role of N‐butyl‐deoxynojirimycin therapy ⬇️

scientific article published on July 20, 2012

Oxidative stress in phenylketonuria: what is the evidence?

scientific article published on 23 April 2011

Oxidative stress in plasma from maple syrup urine disease patients during treatment.

scientific article published on 17 November 2007

Oxidative stress induction by cis-4-decenoic acid: relevance for MCAD deficiency.

scientific article published in November 2007

Oxidative stress is induced in female carriers of X-linked adrenoleukodystrophy

scientific article published on 2 October 2007

Pathogenesis of brain damage in glutaric acidemia type I: Lessons from the genetic mice model

scientific article published on 21 May 2019

Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease

scientific article published on 15 April 2012

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

scientific article published on 30 April 2019

Prevention by L-carnitine of DNA damage induced by propionic and L-methylmalonic acids in human peripheral leukocytes in vitro

scientific article published on 24 July 2010

Pristanic acid promotes oxidative stress in brain cortex of young rats: a possible pathophysiological mechanism for brain damage in peroxisomal disorders

scientific article published on 15 January 2011

Pristanic acid provokes lipid, protein, and DNA oxidative damage and reduces the antioxidant defenses in cerebellum of young rats.

scientific article

Proline impairs energy metabolism in cerebral cortex of young rats.

scientific article published in May 2010

Proline induces oxidative stress in cerebral cortex of rats.

scientific article

Proline reduces acetylcholinesterase activity in cerebral cortex of rats

scientific article published in March 2003

Proline reduces creatine kinase activity in the brain cortex of rats.

scientific article published in August 2003

Promotion of lipid and protein oxidative damage in rat brain by ethylmalonic acid

scientific article published on 16 September 2009

Promotion of oxidative stress by L-tryptophan in cerebral cortex of rats

scientific article published on 23 February 2006

Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester

scientific article published on 30 May 2007

Propionic acid induces cytoskeletal alterations in cultured astrocytes from rat cerebral cortex

scientific article published in March 2006

Propionic and methylmalonic acids increase cAMP levels in slices of cerebral cortex of young rats via adrenergic and glutamatergic mechanisms

scientific article published on 14 January 2005

Protective effect of L-carnitine on Phenylalanine-induced DNA damage

scientific article published on 20 January 2015

Protective effect of antioxidants on brain oxidative damage caused by proline administration

scientific article published in May 2005

Protein and lipid damage in maple syrup urine disease patients: l‐carnitine effect ⬇️

scientific article published on November 5, 2012

Pyruvate kinase activity and δ-aminolevulinic acid dehydratase activity as biomarkers of toxicity in workers exposed to lead.

scientific article published on 5 August 2012

Quinolinic acid reduces the antioxidant defenses in cerebral cortex of young rats

scientific article

Recent Advances in the Pathophysiology of Fatty Acid Oxidation Defects: Secondary Alterations of Bioenergetics and Mitochondrial Calcium Homeostasis Caused by the Accumulating Fatty Acids

scientific article published on 27 November 2020

Reduction of Na(+),K(+)-ATPase activity in hippocampus of rats subjected to chemically induced hyperhomocysteinemia.

scientific article

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 ⬇️

scientific article published on August 29, 2012

Reduction of butyrylcholinesterase activity in plasma from patients with disorders of propionate metabolism is prevented by treatment with L-carnitine and protein restriction

scientific article published on 04 November 2011

Reduction of butyrylcholinesterase activity in rat serum subjected to hyperhomocysteinemia

scientific article published in June 2005

Reduction of energy metabolism in rat hippocampus by arginine administration

scientific article published in September 2003

Reduction of glutamate uptake into cerebral cortex of developing rats by the branched-chain alpha-keto acids accumulating in maple syrup urine disease

scientific article published in April 2004

Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of L-carnitine supplementation

scientific article published on 25 January 2010

Resveratrol protects C6 astrocyte cell line against hydrogen peroxide-induced oxidative stress through heme oxygenase 1

scientific article

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

scientific article

Screening for organic acidurias and aminoacidopathies in high-risk Brazilian patients: Eleven-year experience of a reference center

scientific article published on 11 April 2019

Selective Screening of Fatty Acids Oxidation Defects and Organic Acidemias by Liquid Chromatography/tandem Mass Spectrometry Acylcarnitine Analysis in Brazilian Patients

scientific article published on 01 April 2018

Selective screening for organic acidemias by urine organic acid GC-MS analysis in Brazil: fifteen-year experience

scientific article published in November 2008

Signaling mechanisms downstream of quinolinic acid targeting the cytoskeleton of rat striatal neurons and astrocytes

scientific article published on 13 November 2011

Signaling mechanisms underlying the glioprotective effects of resveratrol against mitochondrial dysfunction.

scientific article published in July 2016

Simvastatin treatment prevents oxidative damage to DNA in whole blood leukocytes of dyslipidemic type 2 diabetic patients

scientific article published in July 2010

Stimulation of lipid peroxidation in vitro in rat brain by the metabolites accumulating in maple syrup urine disease

scientific article published in March 2002

Striatal neuronal death mediated by astrocytes from the Gcdh-/- mouse model of glutaric acidemia type I.

scientific article

Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver.

scientific article

Sulfite disrupts brain mitochondrial energy homeostasis and induces mitochondrial permeability transition pore opening via thiol group modification.

scientific article published on 2 May 2014

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

scientific article published in March 2007

The Role of Oxidative Stress and Bioenergetic Dysfunction in Sulfite Oxidase Deficiency: Insights from Animal Models

scientific article published on 05 December 2018

The effect of Lorenzo's oil on oxidative stress in X-linked adrenoleukodystrophy

scientific article

The effects of the interactions between amino acids on pyruvate kinase activity from the brain cortex of young rats.

scientific article published in October 2005

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

scientific article published on 03 September 2020

The molecular landscape of propionic acidemia and methylmalonic aciduria in Latin America

scientific article published on 15 June 2010

Toxic Synergism Between Quinolinic Acid and Glutaric Acid in Neuronal Cells Is Mediated by Oxidative Stress: Insights to a New Toxic Model.

scientific article

Toxicity of octanoate and decanoate in rat peripheral tissues: evidence of bioenergetic dysfunction and oxidative damage induction in liver and skeletal muscle

scientific article published on 21 October 2011

Tryptophan administration induces oxidative stress in brain cortex of rats

scientific article published on 19 April 2008

Tryptophan reduces creatine kinase activity in the brain cortex of rats

scientific article published in April 2004

Tyrosine administration decreases glutathione and stimulates lipid and protein oxidation in rat cerebral cortex.

scientific article published on 18 August 2009

Tyrosine promotes oxidative stress in cerebral cortex of young rats.

scientific article published on 28 May 2008

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

scientific article

Urinary biomarkers of oxidative damage in Maple syrup urine disease: the L-carnitine role

scientific article published on 10 February 2015

Urinary biomarkers of oxidative stress and plasmatic inflammatory profile in phenylketonuric treated patients

scientific article published on 8 October 2015

alpha-Ketoisocaproic acid regulates phosphorylation of intermediate filaments in postnatal rat cortical slices through ionotropic glutamatergic receptors

scientific article published in December 2002

alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats.

scientific article published in June 2005

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

scientific article published on 27 May 2016

cis-4-decenoic acid provokes mitochondrial bioenergetic dysfunction in rat brain

scientific article published on 9 June 2010

d-Serine administration provokes lipid oxidation and decreases the antioxidant defenses in rat striatum

scientific article published on 20 March 2010

l-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload

scientific article published on 08 June 2019

α-Ketoadipic Acid and α-Aminoadipic Acid Cause Disturbance of Glutamatergic Neurotransmission and Induction of Oxidative Stress In Vitro in Brain of Adolescent Rats

scientific article

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