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

New targets for monitoring and therapy in Barth syndrome

(2016) W. Reid Thompson et al.   GENETICS IN MEDICINE

Activating PPAR  Prevents Post-Ischemic Contractile Dysfunction in Hypertrophied Neonatal Hearts

(2015) V. H. Lam et al.   CIRCULATION RESEARCH

Cardiac Metabolic Pathways Affected in the Mouse Model of Barth Syndrome

(2015) Yan Huang et al.   PLoS One

Mapping Genetic Contributions to Cardiac Pathology Induced by Beta-Adrenergic Stimulation in MiceCLINICAL PERSPECTIVE

(2014) Christoph D. Rau et al.   Circulation-Cardiovascular Genetics

No effect of bezafibrate in patients with CPTII and VLCAD deficiencies

(2014) Mette Cathrine Ørngreen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Bezafibrate in skeletal muscle fatty acid oxidation disorders: A randomized clinical trial

(2014) M. C. Orngreen et al.   NEUROLOGY

Barth syndrome

(2013) John L. Jefferies   AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS

Cardiac-specific VLCAD deficiency induces dilated cardiomyopathy and cold intolerance

(2013) Dingding Xiong et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Myocardial energy shortage and unmet anaplerotic needs in the fasted long-chain acyl-CoA dehydrogenase knockout mouse

(2013) Adrianus J. Bakermans et al.   CARDIOVASCULAR RESEARCH

Mitochondrial Complex I Deficiency Increases Protein Acetylation and Accelerates Heart Failure

(2013) Georgios Karamanlidis et al.   Cell Metabolism

Dysfunctional cardiac mitochondrial bioenergetic, lipidomic, and signaling in a murine model of Barth syndrome

(2013) Michael A. Kiebish et al.   JOURNAL OF LIPID RESEARCH

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

Bezafibrate administration improves behavioral deficits and tau pathology in P301S mice

(2012) Magali Dumont et al.   HUMAN MOLECULAR GENETICS

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

(2012) Natalie Noe et al.   MITOCHONDRION

Left Ventricular Noncompaction Cardiomyopathy in Barth Syndrome: An Example of an Undulating Cardiac Phenotype Necessitating Mechanical Circulatory Support as a Bridge to Transplantation

(2012) Samuel P. Hanke et al.   PEDIATRIC CARDIOLOGY

Long-Term Bezafibrate Treatment Improves Skin and Spleen Phenotypes of the mtDNA Mutator Mouse

(2012) Lloye M. Dillon et al.   PLoS One

Tafazzin Knockdown in Mice Leads to a Developmental Cardiomyopathy With Early Diastolic Dysfunction Preceding Myocardial Noncompaction

(2012) Colin K. L. Phoon et al.   Journal of the American Heart Association

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

(2011) Carlo Viscomi et al.   Cell 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

Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice

(2011) Shuichi Yatsuga et al.   HUMAN MOLECULAR GENETICS

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

Pretreatment with quercetin ameliorates lipids, lipoproteins and marker enzymes of lipid metabolism in isoproterenol treated cardiotoxic male Wistar rats

(2010) Ponnian Stanely Mainzen Prince et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Characterization of a Transgenic Short Hairpin RNA-Induced Murine Model of Tafazzin Deficiency

(2010) Meghan S. Soustek et al.   HUMAN GENE THERAPY

Cardiac and Skeletal Muscle Defects in a Mouse Model of Human Barth Syndrome

(2010) Devrim Acehan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Barth syndrome: an X-linked cause of fetal cardiomyopathy and stillbirth

(2010) C. G. Steward et al.   PRENATAL DIAGNOSIS

Bezafibrate at Clinically Relevant Doses Decreases Serum/Liver Triglycerides via Down-Regulation of Sterol Regulatory Element-Binding Protein-1c in Mice: A Novel Peroxisome Proliferator-Activated Receptor  -Independent Mechanism

(2009) T. Nakajima et al.   MOLECULAR PHARMACOLOGY

Cold acclimation induces physiological cardiac hypertrophy and increases assimilation of triacylglycerol metabolism through lipoprotein lipase

(2008) Y. Cheng et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

RETRACTED: Activation of the PPAR/PGC-1α Pathway Prevents a Bioenergetic Deficit and Effectively Improves a Mitochondrial Myopathy Phenotype

(2008) Tina Wenz et al.   Cell Metabolism