Disturbance of Mitochondrial Dynamics, Endoplasmic Reticulum–Mitochondria Crosstalk, Redox Homeostasis, and Inflammatory Response in the Brain of Glutaryl-CoA Dehydrogenase-Deficient Mice: Neuroprotective Effects of Bezafibrate

The first knock-in rat model for glutaric aciduria type I allows further insights into pathophysiology in brain and periphery

(2021) Mary Gonzalez Melo et al.   MOLECULAR GENETICS AND METABOLISM

Protective effects of L-carnitine on behavioral alterations and neuroinflammation in striatum of glutaryl-COA dehydrogenase deficient mice

(2021) Gilian Guerreiro et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Neuronal Death, Glial Reactivity, Microglia Activation, Oxidative Stress and Bioenergetics Impairment Caused by Intracerebroventricular Administration of D-2-hydroxyglutaric Acid to Neonatal Rats

(2021) Rafael Teixeira Ribeiro et al.   NEUROSCIENCE

Emerging roles of oxidative stress in brain aging and Alzheimer's disease

(2021) Andra Ionescu-Tucker et al.   NEUROBIOLOGY OF AGING

Nuclear Factor Erythroid-2-Related Factor 2 Signaling in the Neuropathophysiology of Inherited Metabolic Disorders

(2021) Bianca Seminotti et al.   Frontiers in Cellular Neuroscience

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

(2020) Gilian Guerreiro et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

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

(2020) Nevton Teixeira da Rosa-Junior et al.   BIOCHIMIE

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

(2020) Moacir Wajner et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

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

(2020) Bianca Seminotti et al.   NEUROTOXICITY RESEARCH

Sustained glial reactivity induced by glutaric acid may be the trigger to learning delay in early and late phases of development: Involvement of p75NTR receptor and protection by N-acetylcysteine

(2020) Fernanda Silva Rodrigues et al.   BRAIN RESEARCH

The C/EBP Homologous Protein (CHOP) Transcription Factor Functions in Endoplasmic Reticulum Stress-Induced Apoptosis and Microbial Infection

(2019) Hai Hu et al.   Frontiers in Immunology

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

(2019) Nevton Teixeira da Rosa-Junior et al.   NEUROTOXICITY RESEARCH

Neurological manifestations of organic acidurias

(2019) Moacir Wajner   Nature Reviews Neurology

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

(2019) Moacir Wajner et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Endoplasmic reticulum–mitochondria crosstalk: from junction to function across neurological disorders

(2019) Pabbala Veeresh et al.   Annals of the New York Academy of Sciences

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

(2019) Gilian Guerreiro et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

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

(2019) Alexandre Umpierrez Amaral et al.   NEUROCHEMISTRY INTERNATIONAL

Oxidative stress in urea cycle disorders: Findings from clinical and basic research

(2018) Belisa Parmeggiani et al.   CLINICA CHIMICA ACTA

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

(2018) Belisa Parmeggiani et al.   MOLECULAR NEUROBIOLOGY

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

(2018) Silvia Olivera-Bravo et al.   MOLECULAR NEUROBIOLOGY

Endoplasmic Reticulum–Mitochondrial Contactology: Structure and Signaling Functions

(2018) György Csordás et al.   TRENDS IN CELL BIOLOGY

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

(2018) Gilian Guerreiro et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

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

(2018) Bianca Seminotti et al.   MOLECULAR NEUROBIOLOGY

Involvement of the Cholinergic Parameters and Glial Cells in Learning Delay Induced by Glutaric Acid: Protection by N-Acetylcysteine

(2018) Fernanda Silva Rodrigues et al.   MOLECULAR NEUROBIOLOGY

NRF2 and NF-қB interplay in cerebrovascular and neurodegenerative disorders: Molecular mechanisms and possible therapeutic approaches

(2018) Farzane Sivandzade et al.   Redox Biology

Mitochondrial dynamics in neuronal injury, development and plasticity

(2017) Kyle H. Flippo et al.   JOURNAL OF CELL SCIENCE

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

Morphological control of mitochondrial bioenergetics

(2015) Yisang Yoon   Frontiers in Bioscience-Landmark

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

(2015) Guilhian Leipnitz et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Mitochondrial trifunctional protein deficiency in human cultured fibroblasts: effects of bezafibrate

(2015) Fatima Djouadi et al.   JOURNAL OF INHERITED METABOLIC DISEASE

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

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

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

(2014) Estela Natacha Brandt Busanello et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

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

(2014) Bianca Seminotti et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Crosstalk Between Endoplasmic Reticulum Stress, Oxidative Stress, and Autophagy: Potential Therapeutic Targets for Acute CNS Injuries

(2014) Venkata Prasuja Nakka et al.   MOLECULAR NEUROBIOLOGY

PPARγ and PGC-1α as Therapeutic Targets in Parkinson’s

(2014) Juan Carlos Corona et al.   NEUROCHEMICAL RESEARCH

Mitochondrial Morphological Features Are Associated with Fission and Fusion Events

(2014) Laura M. Westrate et al.   PLoS One

Glutaric Acid-Mediated Apoptosis in Primary Striatal Neurons

(2014) Fengyan Tian et al.   Biomed Research International

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

(2013) Graziela S. Ribas et al.   GENE

Mechanisms of Mitochondrial Fission and Fusion

(2013) A. M. van der Bliek et al.   Cold Spring Harbor Perspectives in Biology

The effects of repeated oral gavage on the health of male CD-1 mice

(2012) Regina Arantes-Rodrigues et al.   LAB ANIMAL

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

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

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

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

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

Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease

(2011) Ashu Johri et al.   HUMAN MOLECULAR GENETICS

The unsolved puzzle of neuropathogenesis in glutaric aciduria type I

(2011) Paris Jafari et al.   MOLECULAR GENETICS AND METABOLISM

Compared effects of missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase deficiency: Combined analysis by structural, functional and pharmacological approaches

(2010) Stéphanie Gobin-Limballe et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Long-Term Follow-Up of Bezafibrate Treatment in Patients With the Myopathic Form of Carnitine Palmitoyltransferase 2 Deficiency

(2010) J P Bonnefont et al.   CLINICAL PHARMACOLOGY & THERAPEUTICS

Profiling of oxidative stress in patients with inborn errors of metabolism

(2009) Peter J. Mc Guire et al.   MOLECULAR GENETICS AND METABOLISM

Coupling mitochondrial dysfunction to endoplasmic reticulum stress response: A molecular mechanism leading to hepatic insulin resistance

(2008) J LIM et al.   CELLULAR SIGNALLING

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

(2008) Silvia Olivera et al.   NEUROBIOLOGY OF DISEASE

Modulation of mitochondrial morphology by bioenergetics defects in primary human fibroblasts

(2008) O. Guillery et al.   NEUROMUSCULAR DISORDERS