Moacir Wajner

Moacir Wajner

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Moacir Wajner

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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.

Toxicol In Vitro 2020 Feb 16;62:104665. Epub 2019 Oct 16.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.tiv.2019.104665DOI Listing
February 2020

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

Mitochondrion 2020 Jan 23;50:1-13. Epub 2019 Oct 23.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.mito.2019.09.008DOI Listing
January 2020

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.

Arch Biochem Biophys 2020 Jan 22;679:108206. Epub 2019 Nov 22.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, UFRGS, Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, UFRGS, Porto Alegre, RS, Brazil; Faculdade de Farmácia, UFRGS, Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.abb.2019.108206DOI Listing
January 2020

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

Biochim Biophys Acta Mol Basis Dis 2020 Jan 10;1866(5):165682. Epub 2020 Jan 10.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Departamento de Ciências Biológicas, Universidade Regional Integrada do Alto Uruguai e das Missões, Erechim, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2020.165682DOI Listing
January 2020

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

Int J Dev Neurosci 2019 Nov 21;78:215-221. Epub 2019 May 21.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600, Prédio, 21111, Porto Alegre, RS, 90035-003, Brazil.

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http://dx.doi.org/10.1016/j.ijdevneu.2019.05.005DOI Listing
November 2019

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

Mol Neurobiol 2019 Nov 9. Epub 2019 Nov 9.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12035-019-01825-0DOI Listing
November 2019

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

Neurotox Res 2019 Nov 13. Epub 2019 Nov 13.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, Porto Alegre, RS, CEP 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12640-019-00122-xDOI Listing
November 2019

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

Neurochem Int 2019 10 20;129:104467. Epub 2019 May 20.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.neuint.2019.104467DOI Listing
October 2019

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

Biochim Biophys Acta Mol Basis Dis 2019 Sep 8;1865(9):2420-2427. Epub 2019 Jun 8.

Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em CB:Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035 000 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, 90610-000 Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2019.06.007DOI Listing
September 2019

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

Mol Cell Biochem 2019 Aug 29;458(1-2):99-112. Epub 2019 Apr 29.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s11010-019-03534-7DOI Listing
August 2019

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

Mitochondrion 2019 07 3;47:141-150. Epub 2018 Nov 3.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul, Ramiro Barcelos Street, 2600-Attached, CEP 90035-003 Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Ramiro Barcelos Street, 2600-Attached, CEP 90035-003 Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.mito.2018.11.001DOI Listing
July 2019

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.

Arch Biochem Biophys 2019 Jun 30;668:16-22. Epub 2019 Apr 30.

Programa de Pós-Graduação em Ciências Farmacêuticas, Faculdade de Farmácia, UFRGS, Av. Ipiranga, 27522, CEP, 90610-000, Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, UFRGS, R.Ramiro Barcelos, 2600, CEP, 90035-03, Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, R.Ramiro Barcelos, 2350, CEP, 90035-003, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.abb.2019.04.008DOI Listing
June 2019

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

Neurotox Res 2019 May 9;35(4):809-822. Epub 2019 Mar 9.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street - Attached, Porto Alegre, Rio Grande do Sul, CEP 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12640-019-00019-9DOI Listing
May 2019

Neurological manifestations of organic acidurias.

Authors:
Moacir Wajner

Nat Rev Neurol 2019 May;15(5):253-271

Department of Biochemistry, Institute of Basic Sciences and Health, Federal University of Rio Grande do Sul, Porto Alegre, Brazil.

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http://dx.doi.org/10.1038/s41582-019-0161-9DOI Listing
May 2019

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

Mol Neurobiol 2019 Apr 30;56(4):2760-2773. Epub 2018 Jul 30.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12035-018-1275-6DOI Listing
April 2019

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

Genet Mol Biol 2019 11;42(1 suppl 1):178-185. Epub 2019 Apr 11.

Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1590/1678-4685-GMB-2018-0105DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6687352PMC
April 2019

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

Neuroscience 2019 03 19;401:84-95. Epub 2019 Jan 19.

Laboratorio de Aminoácidos Excitadores, Instituto Nacional de Neurología y Neurocirugía, S.S.A., Mexico City, Mexico. Electronic address:

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http://dx.doi.org/10.1016/j.neuroscience.2019.01.014DOI Listing
March 2019

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

Neurotox Res 2019 Feb 5;35(2):484-494. Epub 2018 Dec 5.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Departamento de Bioquímica, ICBS, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, Porto Alegre, RS, 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12640-018-9986-zDOI Listing
February 2019

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

Mol Neurobiol 2019 Jan 19;56(1):29-38. Epub 2018 Apr 19.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos No. 2600 - Attached, Porto Alegre, RS, 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12035-018-1074-0DOI Listing
January 2019

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

Mol Neurobiol 2019 Jan 19;56(1):648-657. Epub 2018 May 19.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, CEP 900035-003, Brazil.

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http://dx.doi.org/10.1007/s12035-018-1077-xDOI Listing
January 2019

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

Neurotox Res 2018 Oct 28;34(3):693-705. Epub 2018 Jul 28.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, Porto Alegre, RS, 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12640-018-9934-yDOI Listing
October 2018

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

J Cell Biochem 2018 09 20;119(9):7678-7686. Epub 2018 Jun 20.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.

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http://doi.wiley.com/10.1002/jcb.27115
Publisher Site
http://dx.doi.org/10.1002/jcb.27115DOI Listing
September 2018

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.

Mol Neurobiol 2018 Jul 3;55(7):5868-5878. Epub 2017 Nov 3.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12035-017-0804-zDOI Listing
July 2018

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

Mol Neurobiol 2018 Jun 24;55(6):5101-5110. Epub 2017 Aug 24.

Departamento de Bioquímica, Universidade Federal do Rio Grande do Sul, Instituto de Ciências Básicas da Saúde, Rua Ramiro Barcelos 2600, Porto Alegre, RS, CEP 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12035-017-0711-3DOI Listing
June 2018

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

Mol Neurobiol 2018 Jun 21;55(6):5362-5376. Epub 2017 Sep 21.

Laboratorio de Aminoácidos Excitadores, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, SSA, Insurgentes Sur 3877, 14269, Mexico City, Mexico.

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http://dx.doi.org/10.1007/s12035-017-0761-6DOI Listing
June 2018

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

Mol Neurobiol 2018 May 5;55(5):4068-4077. Epub 2017 Jun 5.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600, Anexo, Porto Alegre, RS, CEP 90.035-003, Brazil.

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http://dx.doi.org/10.1007/s12035-017-0626-zDOI Listing
May 2018

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

Neurotox Res 2018 04 12;33(3):593-606. Epub 2017 Dec 12.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://link.springer.com/10.1007/s12640-017-9848-0
Publisher Site
http://dx.doi.org/10.1007/s12640-017-9848-0DOI Listing
April 2018

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.

Neurotox Res 2018 04 6;33(3):681-692. Epub 2018 Feb 6.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12640-018-9874-6DOI Listing
April 2018

Metabolite accumulation in VLCAD deficiency markedly disrupts mitochondrial bioenergetics and Ca homeostasis in the heart.

FEBS J 2018 04 25;285(8):1437-1455. Epub 2018 Mar 25.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.

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http://dx.doi.org/10.1111/febs.14419DOI Listing
April 2018

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

Arch Med Res 2018 04 16;49(3):205-212. Epub 2018 Aug 16.

Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Biológicas, Bioquímica, Faculdade de Farmácia, UFRGS, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1016/j.arcmed.2018.08.004DOI Listing
April 2018

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

Mol Neurobiol 2018 01 3;55(1):741-750. Epub 2017 Jan 3.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal de Rio Grande do Sul, Rua Ramiro Barcelos N° 2600 - Attached, Porto Alegre, RS, CEP: 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12035-016-0319-zDOI Listing
January 2018

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

Cell Mol Neurobiol 2017 Nov 3;37(8):1477-1485. Epub 2017 Mar 3.

Departamento de Análises, Faculdade de Farmácia, UFRGS, Avenida Ipiranga 2752, Porto Alegre, RS, 90610-000, Brazil.

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http://dx.doi.org/10.1007/s10571-017-0478-0DOI Listing
November 2017

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

Epilepsia 2017 10 1;58(10):1771-1781. Epub 2017 Aug 1.

Postgraduate Program in Biological Sciences: Biochemistry, Department of Biochemistry, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.

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http://doi.wiley.com/10.1111/epi.13862
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http://dx.doi.org/10.1111/epi.13862DOI Listing
October 2017

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

Neurochem Int 2017 Sep 9;108:133-145. Epub 2017 Mar 9.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.neuint.2017.03.005DOI Listing
September 2017

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.

Biochim Biophys Acta Mol Basis Dis 2017 09 18;1863(9):2135-2148. Epub 2017 May 18.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003 Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003 Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2017.05.019DOI Listing
September 2017

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.

Biochim Biophys Acta Mol Basis Dis 2017 09 15;1863(9):2192-2201. Epub 2017 Jun 15.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003 Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003 Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2017.06.007DOI Listing
September 2017

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

Mol Neurobiol 2017 08 29;54(6):4496-4506. Epub 2016 Jun 29.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, UFRGS, Rua Ramiro Barcelos 2600, CEP 90.035-003, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12035-016-9984-1DOI Listing
August 2017

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.

Toxicol In Vitro 2017 Aug 7;42:47-53. Epub 2017 Apr 7.

Programa de Pós-Graduação em Ciências Farmacêuticas, Faculdade de Farmácia, UFRGS, Av. Ipiranga, 2752, CEP 90610-000 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, UFRGS, R. Ramiro Barcelos, 2600, CEP 90035-003 Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, R. Ramiro Barcelos, 2350, CEP 90035-003 Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.tiv.2017.04.006DOI Listing
August 2017

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

Neurotox Res 2017 Aug 17;32(2):264-275. Epub 2017 Apr 17.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600 - Attached, Porto Alegre, RS, CEP: 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12640-017-9732-yDOI Listing
August 2017

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

Biochim Biophys Acta 2016 Nov 12;1862(11):2063-2074. Epub 2016 Aug 12.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2016.08.007DOI Listing
November 2016

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

Mol Neurobiol 2016 11 26;53(9):5864-5875. Epub 2015 Oct 26.

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos No. 2600, 90035-003, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12035-015-9493-7DOI Listing
November 2016

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.

Mol Neurobiol 2016 11 25;53(9):6459-6475. Epub 2015 Nov 25.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12035-015-9548-9DOI Listing
November 2016

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

Toxicol In Vitro 2016 Oct 29;36:1-9. Epub 2016 Jun 29.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.tiv.2016.06.007DOI Listing
October 2016

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.

Neurochem Res 2016 Sep 9;41(9):2190-8. Epub 2016 May 9.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos No 2600 - Anexo, Porto Alegre, RS, 90035-003, Brazil.

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http://dx.doi.org/10.1007/s11064-016-1933-xDOI Listing
September 2016

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.

Biochim Biophys Acta 2016 09 27;1857(9):1363-1372. Epub 2016 May 27.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbabio.2016.05.007DOI Listing
September 2016

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

Biochim Biophys Acta 2016 09 1;1862(9):1827-38. Epub 2016 Jul 1.

Departamento de Bioquímica, Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2016.06.018DOI Listing
September 2016

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.

Free Radic Res 2016 Sep 23;50(9):997-1010. Epub 2016 Aug 23.

a Programa de Pós-Graduação em Ciências Biológicas: Bioquímica , Instituto de Ciências Básicas da Saúde , UFRGS , Porto Alegre, RS , Brazil ;

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http://dx.doi.org/10.1080/10715762.2016.1214952DOI Listing
September 2016

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.

Mol Neurobiol 2016 08 23;53(6):3586-3595. Epub 2015 Jun 23.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos No 2600 - Anexo, 90035-003, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12035-015-9289-9DOI Listing
August 2016

Oxidative Stress in Patients with X-Linked Adrenoleukodystrophy.

Cell Mol Neurobiol 2016 May 14;36(4):497-512. Epub 2015 Jul 14.

Programa de Pós-Graduação em Ciências Farmacêuticas, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s10571-015-0234-2DOI Listing
May 2016

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

J Neurochem 2016 Apr 15;137(1):62-75. Epub 2016 Feb 15.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal de Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1111/jnc.13544DOI Listing
April 2016

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

Int J Dev Neurosci 2015 Dec 8;47(Pt B):259-65. Epub 2015 Oct 8.

Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, CEP 90610-000 Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, Rua Ramiro Barcelos, 2350, CEP 90035-003 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Biológicas, Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, CEP 90035-003 Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.ijdevneu.2015.10.001DOI Listing
December 2015

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.

J Neurol Sci 2015 Dec 28;359(1-2):133-40. Epub 2015 Oct 28.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2015.10.043DOI Listing
December 2015

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

J Inherit Metab Dis 2015 Nov 4;38(6):1021-8. Epub 2015 Jun 4.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600 - Attached, CEP 90035-003, Alegre Porto, RS, Brazil.

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http://dx.doi.org/10.1007/s10545-015-9863-3DOI Listing
November 2015

Mitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studies.

Biosci Rep 2015 Nov 20;36(1):e00281. Epub 2015 Nov 20.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal de Rio Grande do Sul, Porto Alegre, RS 90035-003, Brazil.

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http://dx.doi.org/10.1042/BSR20150240DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4718505PMC
November 2015

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

Metab Brain Dis 2015 Oct 24;30(5):1167-74. Epub 2015 May 24.

Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903, Porto Alegre, RS, Brazil,

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http://dx.doi.org/10.1007/s11011-015-9686-9DOI Listing
October 2015

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

Brain Res 2015 Sep 18;1620:116-29. Epub 2015 May 18.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00068993150039
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http://dx.doi.org/10.1016/j.brainres.2015.05.013DOI Listing
September 2015

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

Metab Brain Dis 2015 Aug 20;30(4):925-33. Epub 2015 Jan 20.

Serviço de Genética Médica, HCPA, Rua Ramiro Barcelos, 2350, CEP 90035-003, Porto Alegre, RS, Brazil,

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http://dx.doi.org/10.1007/s11011-015-9649-1DOI Listing
August 2015

Ornithine In Vivo Administration Disrupts Redox Homeostasis and Decreases Synaptic Na(+), K (+)-ATPase Activity in Cerebellum of Adolescent Rats: Implications for the Pathogenesis of Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH) Syndrome.

Cell Mol Neurobiol 2015 Aug 13;35(6):797-806. Epub 2015 Mar 13.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos No 2600 - Anexo, Porto Alegre, RS, CEP 90035-003, Brazil.

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http://dx.doi.org/10.1007/s10571-015-0173-yDOI Listing
August 2015

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.

Biochim Biophys Acta 2015 Jun-Jul;1847(6-7):620-8. Epub 2015 Apr 11.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbabio.2015.04.003DOI Listing
August 2015

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

Metab Brain Dis 2015 Aug 5;30(4):1055-62. Epub 2015 May 5.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600 - Attached, CEP 90035-003, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s11011-015-9675-zDOI Listing
August 2015

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

Free Radic Biol Med 2015 Jun 17;83:201-13. Epub 2015 Feb 17.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.freeradbiomed.2015.02.008DOI Listing
June 2015

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.

Biochim Biophys Acta 2015 May 10;1852(5):759-67. Epub 2015 Jan 10.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2015.01.003DOI Listing
May 2015

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

Int J Dev Neurosci 2015 May 10;42:10-4. Epub 2015 Feb 10.

Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035-000 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, 90610-000 Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1016/j.ijdevneu.2015.02.003DOI Listing
May 2015

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

Int J Dev Neurosci 2015 May 13;42:68-75. Epub 2015 Mar 13.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 - Anexo - CEP, 90035-003 Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.ijdevneu.2015.03.005DOI Listing
May 2015

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

Mutat Res 2015 May 28;775:43-7. Epub 2015 Mar 28.

Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em CB:Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035 000, Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, 90610-000 Porto Alegre, RS, Brazil.

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https://linkinghub.elsevier.com/retrieve/pii/S00275107150006
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http://dx.doi.org/10.1016/j.mrfmmm.2015.03.008DOI Listing
May 2015

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

Mol Cell Biochem 2015 Apr 4;402(1-2):149-55. Epub 2015 Jan 4.

Programa de Pós-Graduação Ciências Farmacêuticas da Universidade Federal do Rio Grande do Sul (UFRGS), Ipiranga 2752, Porto Alegre, RS, 90610-000, Brazil,

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http://dx.doi.org/10.1007/s11010-014-2322-xDOI Listing
April 2015

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.

PLoS One 2014 4;9(3):e90477. Epub 2014 Mar 4.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0090477PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3942441PMC
February 2015

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

Cerebellum 2014 Dec;13(6):751-9

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 - Anexo, Porto Alegre, RS, CEP 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12311-014-0593-0DOI Listing
December 2014

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.

J Neurol Sci 2014 Nov 16;346(1-2):260-7. Epub 2014 Sep 16.

Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2014.09.003DOI Listing
November 2014

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

Mol Cell Biochem 2014 Oct 18;395(1-2):125-34. Epub 2014 Jun 18.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal de Rio Grande do Sul, Rua Ramiro Barcelos N° 2600 - Attached, Porto Alegre, RS, CEP 90035-003, Brazil.

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http://dx.doi.org/10.1007/s11010-014-2118-zDOI Listing
October 2014

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

J Inherit Metab Dis 2014 Sep 13;37(5):783-9. Epub 2014 Mar 13.

Section of Metabolic Diseases, University Medical Center Groningen, University of Groningen, 9700, RB, Groningen, The Netherlands,

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http://dx.doi.org/10.1007/s10545-014-9700-0DOI Listing
September 2014

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

Biochim Biophys Acta 2014 Sep 2;1842(9):1413-22. Epub 2014 May 2.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003 Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2014.04.022DOI Listing
September 2014

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?

Biochim Biophys Acta 2014 Sep 16;1842(9):1658-67. Epub 2014 Jun 16.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2014.06.011DOI Listing
September 2014

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.

J Neurol Sci 2014 Sep 22;344(1-2):105-13. Epub 2014 Jun 22.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2014.06.034DOI Listing
September 2014

Ethylmalonic acid induces permeability transition in isolated brain mitochondria.

Neurotox Res 2014 Aug 21;26(2):168-78. Epub 2014 Feb 21.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal de Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, Porto Alegre, RS, 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12640-014-9460-5DOI Listing
August 2014

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

Gene 2014 Apr 15;539(2):270-4. Epub 2014 Feb 15.

Programa de Pós-Graduação em Ciências Biológicas, Bioquímica da Universidade Federal do Rio Grande do Sul (UFRGS), Ramiro Barcelos 2700, Porto Alegre, RS 90035-000, Brazil; Serviço de Genética Médica do Hospital de Clínicas de Porto Alegre, Ramiro Barcelos 2350, Porto Alegre, RS 90035-903, Brazil.

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http://dx.doi.org/10.1016/j.gene.2014.02.015DOI Listing
April 2014

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

FEBS J 2014 Apr 13;281(8):2061-73. Epub 2014 Mar 13.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, UFRGS, Porto Alegre, RS, Brasil.

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http://dx.doi.org/10.1111/febs.12762DOI Listing
April 2014

Neurological damage in MSUD: the role of oxidative stress.

Cell Mol Neurobiol 2014 Mar 13;34(2):157-65. Epub 2013 Nov 13.

Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcelos, 2350, Porto Alegre, RS, CEP 90035-903, Brazil,

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http://dx.doi.org/10.1007/s10571-013-0002-0DOI Listing
March 2014

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

PLoS One 2013 15;8(5):e64372. Epub 2013 May 15.

Departamento de Bioquímica, Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0064372PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3654976PMC
January 2014

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

Gene 2014 Jan 19;533(2):469-76. Epub 2013 Oct 19.

Federal University of Rio Grande do Sul, Brazil; Serviço de Genética Médica, HCPA, Ramiro Barcelos 2350, Porto Alegre, RS 90035-903, Brazil.

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http://dx.doi.org/10.1016/j.gene.2013.10.017DOI Listing
January 2014

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

Life Sci 2014 Jan 8;94(1):67-73. Epub 2013 Nov 8.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Serviço de Genética Médica do Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. Electronic address:

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http://dx.doi.org/10.1016/j.lfs.2013.10.032DOI Listing
January 2014

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

Gene 2013 Dec 11;531(2):191-8. Epub 2013 Sep 11.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul (UFRGS), Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003 Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1016/j.gene.2013.09.018DOI Listing
December 2013

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

Neurotox Res 2013 Nov 3;24(4):502-11. Epub 2013 May 3.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal de Rio Grande do Sul, Rua Ramiro Barcelos N° 2600-Anexo, Porto Alegre, RS, CEP 90035-003, Brazil.

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http://dx.doi.org/10.1007/s12640-013-9396-1DOI Listing
November 2013

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

Life Sci 2013 Aug 24;93(4):161-8. Epub 2013 Jun 24.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1016/j.lfs.2013.06.013DOI Listing
August 2013

Disruption of mitochondrial homeostasis by phytanic acid in cerebellum of young rats.

Cerebellum 2013 Jun;12(3):362-9

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s12311-012-0426-yDOI Listing
June 2013

Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria.

J Bioenerg Biomembr 2013 Feb 13;45(1-2):47-57. Epub 2012 Oct 13.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003 Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s10863-012-9481-9DOI Listing
February 2013

Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect.

Int J Dev Neurosci 2013 Feb 5;31(1):21-4. Epub 2012 Nov 5.

Programa de Pós-Graduação em CB:Bioquímica e Departamento de Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035-000 Porto Alegre, RS, Brazil.

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https://linkinghub.elsevier.com/retrieve/pii/S07365748120058
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http://dx.doi.org/10.1016/j.ijdevneu.2012.10.109DOI Listing
February 2013

Marked inhibition of Na+, K(+)- ATPase activity and the respiratory chain by phytanic acid in cerebellum from young rats: possible underlying mechanisms of cerebellar ataxia in Refsum disease.

J Bioenerg Biomembr 2013 Feb 15;45(1-2):137-44. Epub 2012 Nov 15.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 - Anexo, CEP 90035-003 Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1007/s10863-012-9491-7DOI Listing
February 2013

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

Life Sci 2013 Feb 28;92(2):137-42. Epub 2012 Nov 28.

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

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http://dx.doi.org/10.1016/j.lfs.2012.11.013DOI Listing
February 2013