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

Globotriaosylsphingosine induces oxidative DNA damage in cultured kidney cells

(2017) Giovana Brondani Biancini et al.   NEPHROLOGY

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

(2017) Daiane Grigolo Bardemaker Rodrigues et al.   TOXICOLOGY IN VITRO

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

(2016) Marília Danyelle Nunes Rodrigues et al.   MOLECULAR NEUROBIOLOGY

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

(2015) Alexandre Umpierrez Amaral et al.   BRAIN RESEARCH

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

(2015) Marion Deon et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Urinary biomarkers of oxidative damage in Maple syrup urine disease: The l-carnitine role

(2015) Gilian Guerreiro et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

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

(2015) Marília Danyelle Nunes Rodrigues et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

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

(2015) Caroline Paula Mescka et al.   METABOLIC BRAIN DISEASE

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

(2015) Marion Deon et al.   METABOLIC BRAIN DISEASE

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

(2015) Mariana dos Santos Mello et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

L-carnitine Prevents Oxidative Stress in the Brains of Rats Subjected to a Chemically Induced Chronic Model of MSUD

(2015) Caroline Paula Mescka et al.   MOLECULAR NEUROBIOLOGY

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

(2015) Bianca Seminotti et al.   MOLECULAR NEUROBIOLOGY

Toxic synergism between quinolinic acid and organic acids accumulating in glutaric acidemia type I and in disorders of propionate metabolism in rat brain synaptosomes: Relevance for metabolic acidemias

(2015) A.L. Colín-González et al.   NEUROSCIENCE

The role of oxidative stress during inflammatory processes

(2013) Jérôme Lugrin et al.   BIOLOGICAL CHEMISTRY

Glutaric aciduria type I: Outcome of patients with early- versus late-diagnosis

(2013) Ma Luz Couce et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

Newborn screening by tandem mass spectrometry for glutaric aciduria type 1: a cost-effectiveness analysis

(2013) Johannes Pfeil et al.   Orphanet Journal of Rare Diseases

Dietary practices in glutaric aciduria type 1 over 16 years

(2012) H. Gokmen-Ozel et al.   JOURNAL OF HUMAN NUTRITION AND DIETETICS

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

(2012) Alexandre Umpierrez Amaral et al.   MOLECULAR GENETICS AND METABOLISM

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

(2012) Bianca Seminotti et al.   MOLECULAR GENETICS AND METABOLISM

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

(2011) Graziela S. Ribas et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Cytokines and brain excitability

(2011) Michael A. Galic et al.   FRONTIERS IN NEUROENDOCRINOLOGY

Use of guidelines improves the neurological outcome in glutaric aciduria type I

(2010) Jana Heringer et al.   ANNALS OF NEUROLOGY

Studies on Plasmalogen-Selective Phospholipase A2 in Brain

(2010) Akhlaq A. Farooqui   MOLECULAR NEUROBIOLOGY

l-carnitine protects plasma components against oxidative alterations

(2010) Joanna Kolodziejczyk et al.   NUTRITION

Functional Aspects of Redox Control During Neuroinflammation

(2009) Sergio Rosales-Corral et al.   ANTIOXIDANTS & REDOX SIGNALING

8-hydroxy-2′ -deoxyguanosine (8-OHdG): A Critical Biomarker of Oxidative Stress and Carcinogenesis

(2009) ATHANASIOS VALAVANIDIS et al.   JOURNAL OF ENVIRONMENTAL SCIENCE AND HEALTH PART C-ENVIRONMENTAL CARCINOGENESIS & ECOTOXICOLOGY REVIEWS

Kinetic characterization of l-[3H]glutamate uptake inhibition and increase oxidative damage induced by glutaric acid in striatal synaptosomes of rats

(2008) Danieli Valnes Magni et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Cytokines and anti-cytokines as therapeutics — An update

(2007) Vandana Tayal et al.   EUROPEAN JOURNAL OF PHARMACOLOGY