Antioxidant system disturbances and mitochondrial dysfunction induced by 3-methyglutaric acid in rat heart are prevented by bezafibrate

Barth syndrome: cardiolipin, cellular pathophysiology, management, and novel therapeutic targets

(2021) Hana M. Zegallai et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

Increased Reactive Oxygen Species–Mediated Ca 2+ /Calmodulin-Dependent Protein Kinase II Activation Contributes to Calcium Handling Abnormalities and Impaired Contraction in Barth Syndrome

(2021) Xujie Liu et al.   CIRCULATION

PI3K/AKT Signal Pathway: A Target of Natural Products in the Prevention and Treatment of Alzheimer’s Disease and Parkinson’s Disease

(2021) Hui-Zhi Long et al.   Frontiers in Pharmacology

Sirtuins as molecular targets, mediators, and protective agents in metal-induced toxicity

(2021) Alexey A. Tinkov et al.   ARCHIVES OF TOXICOLOGY

Treatment of Barth Syndrome by Cardiolipin Manipulation (CARDIOMAN) With Bezafibrate: Protocol for a Randomized Placebo-Controlled Pilot Trial Conducted in the Nationally Commissioned Barth Syndrome Service

(2021) Lucy Dabner et al.   JMIR Research Protocols

Targetable Pathways for Alleviating Mitochondrial Dysfunction in Neurodegeneration of Metabolic and Non-Metabolic Diseases

(2021) Lauren Elizabeth Millichap et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Phenotype and pathology of the dilated cardiomyopathy with ataxia syndrome in children

(2021) Pranav Machiraju et al.   JOURNAL OF INHERITED METABOLIC DISEASE

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

AAV Gene Therapy Prevents and Reverses Heart Failure in a Murine Knockout Model of Barth Syndrome

(2020) Suya Wang et al.   CIRCULATION RESEARCH

PGC-1α-mediated regulation of mitochondrial function and physiological implications

(2020) Jens Frey Halling et al.   Applied Physiology Nutrition and Metabolism

Barth syndrome cardiomyopathy: targeting the mitochondria with elamipretide

(2020) Hani N. Sabbah   HEART FAILURE REVIEWS

The progression of pulmonary arterial hypertension induced by monocrotaline is characterized by lung nitrosative and oxidative stress, and impaired pulmonary artery reactivity

(2020) Alexsandra Zimmer et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

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

Increased mtDNA Abundance and Improved Function in Human Barth Syndrome Patient Fibroblasts Following AAV-TAZ Gene Delivery

(2019) Silveli Suzuki-Hatano et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

A critical appraisal of the tafazzin knockdown mouse model of Barth syndrome: what have we learned about pathogenesis and potential treatments?

(2019) Mindong Ren et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Reversible Mitochondrial Fragmentation in iPSC-Derived Cardiomyocytes from Children with DCMA, a Mitochondrial Cardiomyopathy

(2019) Leili Rohani et al.   CANADIAN JOURNAL OF CARDIOLOGY

Myocardial glucose and fatty acid metabolism is altered and associated with lower cardiac function in young adults with Barth syndrome

(2019) William Todd Cade et al.   JOURNAL OF NUCLEAR CARDIOLOGY

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

(2019) Mateus Struecker da Rosa et al.   NEUROTOXICITY RESEARCH

Inhibition of PRMT5 Attenuates Oxidative Stress-Induced Pyroptosis via Activation of the Nrf2/HO-1 Signal Pathway in a Mouse Model of Renal Ischemia-Reperfusion Injury

(2019) Changhui Diao et al.   Oxidative Medicine and Cellular Longevity

Redox Mechanisms in Neurodegeneration: From Disease Outcomes to Therapeutic Opportunities

(2018) Juan I. Sbodio et al.   ANTIOXIDANTS & REDOX SIGNALING

Peroxisome Proliferator-Activated Receptor Gamma Coactivator-1 Alpha as a Novel Target for Bipolar Disorder and Other Neuropsychiatric Disorders

(2018) Andrew A. Nierenberg et al.   BIOLOGICAL PSYCHIATRY

Addition of Digoxin Improves Cardiac Function in Children With the Dilated Cardiomyopathy With Ataxia Syndrome: A Mitochondrial Cardiomyopathy

(2018) Steven C. Greenway et al.   CANADIAN JOURNAL OF CARDIOLOGY

Overexpression of miR-22 attenuates oxidative stress injury in diabetic cardiomyopathy via Sirt 1

(2018) Qinghui Tang et al.   Cardiovascular Therapeutics

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

(2018) Belisa Parmeggiani et al.   MOLECULAR NEUROBIOLOGY

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

The Effects of PPAR Stimulation on Cardiac Metabolic Pathways in Barth Syndrome Mice

(2018) Caitlin Schafer et al.   Frontiers in Pharmacology

Bezafibrate induces autophagy and improves hepatic lipid metabolism in glycogen storage disease type Ia

(2018) Lauren R Waskowicz et al.   HUMAN MOLECULAR GENETICS

Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency

(2018) Guilhian Leipnitz et al.   Scientific Reports

Heme oxygenase-1 prevents heart against myocardial infarction by attenuating ischemic injury-induced cardiomyocytes senescence

(2018) Huitong Shan et al.   EBioMedicine

Barth Syndrome: Connecting Cardiolipin to Cardiomyopathy

(2017) Nikita Ikon et al.   LIPIDS

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

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

Acquired deficiency of tafazzin in the adult heart: Impact on mitochondrial function and response to cardiac injury

(2016) Karol Szczepanek et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

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

(2016) L. Ferri 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

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

Metabolomics Reveals New Mechanisms for Pathogenesis in Barth Syndrome and Introduces Novel Roles for Cardiolipin in Cellular Function

(2016) Yana Sandlers et al.   PLoS One

Cardiac-specific succinate dehydrogenase deficiency in Barth syndrome

(2015) J. Dudek et al.   EMBO Molecular Medicine

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

(2015) Mateus Struecker da Rosa et al.   METABOLIC BRAIN DISEASE

Chronic Lead Exposure Decreases the Vascular Reactivity of Rat Aortas: The Role of Hydrogen Peroxide

(2015) Karolini Zuqui Nunes et al.   PLoS One

DNAJC19, a Mitochondrial Cochaperone Associated with Cardiomyopathy, Forms a Complex with Prohibitins to Regulate Cardiolipin Remodeling

(2014) Ricarda Richter-Dennerlein et al.   Cell Metabolism

Alterations in Cardiac Structure and Function in Hypertension

(2014) Mário Santos et al.   CURRENT HYPERTENSION REPORTS

Editorial (Thematic Issue: Mitochondrial Biogenesis: Pharmacological Approaches)

(2014) Teresa Valero   CURRENT PHARMACEUTICAL DESIGN

ERK-mediated phosphorylation of TFAM downregulates mitochondrial transcription: Implications for Parkinson's disease

(2014) Kent Z.Q. Wang et al.   MITOCHONDRION

Effects of Bezafibrate Treatment in a Patient and a Carrier With Mutations in the PNPLA2 Gene, Causing Neutral Lipid Storage Disease With Myopathy

(2013) Tineke van de Weijer et al.   CIRCULATION RESEARCH

Low-dose chronic lead exposure increases systolic arterial pressure and vascular reactivity of rat aortas

(2013) Edna Aparecida Silveira et al.   FREE RADICAL BIOLOGY AND MEDICINE

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

Barth syndrome

(2013) Sarah LN Clarke et al.   Orphanet Journal of Rare Diseases

Diminished Exercise Capacity and Mitochondrial bc1 Complex Deficiency in Tafazzin-Knockdown Mice

(2013) Corey Powers et al.   Frontiers in Physiology

ERK and p38 inhibitors attenuate memory deficits and increase CREB phosphorylation and PGC-1α levels in Aβ-injected rats

(2012) Ghorbangol Ashabi et al.   BEHAVIOURAL BRAIN RESEARCH

Mitofusins 1 and 2 Are Essential for Postnatal Metabolic Remodeling in Heart

(2012) Kyriakos N. Papanicolaou et al.   CIRCULATION RESEARCH

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

Bezafibrate for X-Linked Adrenoleukodystrophy

(2012) Marc Engelen et al.   PLoS One

Species Differences in the Effects of Bezafibrate as a Potential Treatment of Mitochondrial Disorders

(2011) Fatima Djouadi et al.   Cell Metabolism

In Vivo Correction of COX Deficiency by Activation of the AMPK/PGC-1α Axis

(2011) Carlo Viscomi et al.   Cell Metabolism

Impairment of both nitric oxide-mediated and EDHF-type relaxation in small mesenteric arteries from rats with streptozotocin-induced diabetes

(2010) CH Leo et al.   BRITISH JOURNAL OF PHARMACOLOGY

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

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

Mitochondrial dysfunction is associated with a pro-apoptotic cellular environment in senescent cardiac muscle

(2009) Vladimir Ljubicic et al.   MECHANISMS OF AGEING AND DEVELOPMENT

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