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

PI3K/Akt/GSK3β induced CREB activation ameliorates arsenic mediated alterations in NMDA receptors and associated signaling in rat hippocampus: Neuroprotective role of curcumin

(2018) Pranay Srivastava et al.   NEUROTOXICOLOGY

Neuroprotective effect of zolpidem against glutamate-induced toxicity is mediated via the PI3K/Akt pathway and inhibited by PK11195

(2018) Maja Jazvinšćak Jembrek et al.   TOXICOLOGY

Barth syndrome cells display widespread remodeling of mitochondrial complexes without affecting metabolic flux distribution

(2018) Iliana A. Chatzispyrou et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: Clinical presentation and outcome in a series of 37 patients

(2017) Sarah Catharina Grünert et al.   MOLECULAR GENETICS AND METABOLISM

The PPAR pan-agonist bezafibrate ameliorates cardiomyopathy in a mouse model of Barth syndrome

(2017) Yan Huang et al.   Orphanet Journal of Rare Diseases

Cilostazol Suppresses Aβ-induced Neurotoxicity in SH-SY5Y Cells through Inhibition of Oxidative Stress and MAPK Signaling Pathway

(2017) Tatsunori Oguchi et al.   Frontiers in Aging Neuroscience

When silence is noise: infantile-onset Barth syndrome caused by a synonymous substitution affecting TAZ gene transcription

(2016) L. Ferri et al.   CLINICAL GENETICS

Mitochondrial epileptic encephalopathy, 3-methylglutaconic aciduria and variable complex V deficiency associated withTIMM50mutations

(2016) M.A. Shahrour et al.   CLINICAL GENETICS

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

(2016) Mateus Struecker da Rosa et al.   FREE RADICAL RESEARCH

Enhanced mitochondrial biogenesis ameliorates disease phenotype in a full-length mouse model of Huntington’s disease

(2016) Abhishek Chandra et al.   HUMAN MOLECULAR GENETICS

First Japanese Case of Carnitine Palmitoyltransferase II Deficiency with the Homozygous Point Mutation S113L

(2016) Atsushi Shima et al.   INTERNAL MEDICINE

Deficiency of HTRA2/Omi is associated with infantile neurodegeneration and 3-methylglutaconic aciduria

(2016) Hanna Mandel et al.   JOURNAL OF MEDICAL GENETICS

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

(2016) Ana Laura Colín-González et al.   NEUROCHEMICAL RESEARCH

CLPB Variants Associated with Autosomal-Recessive Mitochondrial Disorder with Cataract, Neutropenia, Epilepsy, and Methylglutaconic Aciduria

(2015) Carol Saunders et al.   AMERICAN JOURNAL OF HUMAN GENETICS

CLPB Mutations Cause 3-Methylglutaconic Aciduria, Progressive Brain Atrophy, Intellectual Disability, Congenital Neutropenia, Cataracts, Movement Disorder

(2015) Saskia B. Wortmann et al.   AMERICAN JOURNAL OF HUMAN GENETICS

The Neuropsychological profile of patients with 3-Methylglutaconic aciduria type III, Costeff syndrome

(2015) S. Sofer et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART B-NEUROPSYCHIATRIC GENETICS

Mitochondrial dysfunction in primary human fibroblasts triggers an adaptive cell survival program that requires AMPK-α

(2015) Felix Distelmaier et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Very long-chain acyl-coenzyme A dehydrogenase deficiency in Chinese patients: Eight case reports, including one case of prenatal diagnosis

(2015) Xiyuan Li et al.   European Journal of Medical Genetics

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

(2015) Carolina Gonçalves Fernandes 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

Tau and neurodegenerative disease: the story so far

(2015) Khalid Iqbal et al.   Nature Reviews Neurology

Inhibition of Akt Phosphorylation Diminishes Mitochondrial Biogenesis Regulators, Tricarboxylic Acid Cycle Activity and Exacerbates Recognition Memory Deficit in Rat Model of Alzheimer’s Disease

(2014) Fatemeh Shaerzadeh et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Editorial (Thematic Issue: Mitochondrial Biogenesis: Pharmacological Approaches)

(2014) Teresa Valero   CURRENT PHARMACEUTICAL DESIGN

Amelioration of acylcarnitine profile using bezafibrate and riboflavin in a case of adult-onset glutaric acidemia type 2 with novel mutations of the electron transfer flavoprotein dehydrogenase (ETFDH) gene

(2014) Ayako Shioya et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Genomic and non-genomic regulation of PGC1 isoforms by estrogen to increase cerebral vascular mitochondrial biogenesis and reactive oxygen species protection

(2013) Martin F. Kemper et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Redox homeostasis is compromised in vivo by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in rat cerebral cortex and liver

(2013) M. S. da Rosa et al.   FREE RADICAL RESEARCH

Genetic and cellular modifiers of oxidative stress: What can we learn from fatty acid oxidation defects?

(2013) Rikke Katrine Jentoft Olsen et al.   MOLECULAR GENETICS AND METABOLISM

Glutathione redox cycle dysregulation in Huntington’s disease knock-in striatal cells

(2012) Márcio Ribeiro et al.   FREE RADICAL BIOLOGY AND MEDICINE

Bezafibrate administration improves behavioral deficits and tau pathology in P301S mice

(2012) Magali Dumont et al.   HUMAN MOLECULAR GENETICS

Bezafibrate lowers very long-chain fatty acids in X-linked adrenoleukodystrophy fibroblasts by inhibiting fatty acid elongation

(2012) Marc Engelen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

RETRACTED: Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy

(2012) Natalie Noe et al.   MITOCHONDRION

Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: Evaluation by in vitro probe acylcarnitine assay

(2012) Seiji Yamaguchi et al.   MOLECULAR GENETICS AND METABOLISM

Differing short-term neuroprotective effects of the fibrates fenofibrate and bezafibrate in MPTP and 6-OHDA experimental models of Parkinsonʼs disease

(2010) Alexandre Kreisler et al.   BEHAVIOURAL PHARMACOLOGY

The 3-methylglutaconic acidurias: what’s new?

(2010) Saskia B. Wortmann et al.   JOURNAL OF INHERITED METABOLIC DISEASE

3-Methylglutaconic aciduria type I redefined: A syndrome with late-onset leukoencephalopathy

(2010) S. B. Wortmann et al.   NEUROLOGY

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

(2009) Guilhian Leipnitz et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Detection of behavioral alterations and learning deficits in mice lacking synaptophysin

(2009) U. Schmitt et al.   NEUROSCIENCE

Biochemical and genetic analysis of 3-methylglutaconic aciduria type IV: a diagnostic strategy

(2008) Saskia B. Wortmann et al.   BRAIN

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

(2007) Guilhian Leipnitz et al.   JOURNAL OF NEUROSCIENCE RESEARCH