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Elucidating the Beneficial Role of PPAR Agonists in Cardiac Diseases

International journal of molecular sciences, 2018-11, Vol.19 (11), p.3464

2018

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Cardiac metabolic pathways affected in the mouse model of barth syndrome

PloS one, 2015-06, Vol.10 (6), p.e0128561-e0128561

2015

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

Orphanet journal of rare diseases, 2017-03, Vol.12 (1), p.49-49, Article 49

2017

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

Frontiers in pharmacology, 2018-04, Vol.9, p.318-318

2018

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

Journal of inherited metabolic disease, 2016-01, Vol.39 (1), p.47-58

2016

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How many minors are participating in clinical research today? An estimate and important lessons learned

Journal of clinical and translational science, 2021-01, Vol.5 (1), p.e179-e179, Article e179

2021

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A Zebrafish Model of Human Barth Syndrome Reveals the Essential Role of Tafazzin in Cardiac Development and Function

Circulation research, 2006-07, Vol.99 (2), p.201-208

2006

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A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women

The New England journal of medicine, 1999-06, Vol.340 (22), p.1723-1731

1999

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Evaluation of Fellows' CrossTalk Effectiveness in Promoting Transdisciplinary Networking and Research

The Journal of pediatrics, 2017-02, Vol.181, p.5-6.e3

2017

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Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases

American journal of physiology: endocrinology and metabolism, 2003-06, Vol.284 (6), p.E1098-E1105

2003

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Enhancing Pediatric Fellows' Research Training: Development of an Office of Pediatric Clinical Fellowships

The Journal of pediatrics, 2015-09, Vol.167 (3), p.506-507.e1

2015

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Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening

Pediatrics (Evanston), 2008-05, Vol.121 (5), p.e1108-e1114

2008

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Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry

The Journal of pediatrics, 2006-07, Vol.149 (1), p.128-130

2006

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Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein

Muscle & nerve, 2004-01, Vol.29 (1), p.66-72

2004

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Mechanism of Hyperinsulinism in Short-chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency Involves Activation of Glutamate Dehydrogenase

The Journal of biological chemistry, 2010-10, Vol.285 (41), p.31806-31818

2010

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Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death

Circulation (New York, N.Y.), 1999-03, Vol.99 (10), p.1337-1343

1999

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Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice

American journal of physiology. Heart and circulatory physiology, 2007-05, Vol.292 (5), p.H2202-H2211

2007

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Cardiac-specific VLCAD deficiency induces dilated cardiomyopathy and cold intolerance

American journal of physiology. Heart and circulatory physiology, 2014-02, Vol.306 (3), p.H326-H338

2014

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Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice

Pediatric research, 2005-06, Vol.57 (6), p.760-764

2005

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Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card

Pediatrics (Evanston), 2001-07, Vol.108 (1), p.E19-e19

2001

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Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome : Impact of expanded newborn screening on their diagnosis and management

Pediatric research, 2005-05, Vol.57 (5), p.78-86

2005

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MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency

The Journal of pediatrics, 2003-09, Vol.143 (3), p.335-342

2003

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Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to β-subunit mutations

Human mutation, 2003-06, Vol.21 (6), p.598-607

2003

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Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease

JAMA : the journal of the American Medical Association, 2002-11, Vol.288 (17), p.2163

2002

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Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation

Journal of the American College of Cardiology, 2004-07, Vol.44 (2), p.391-397

2004

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Elucidating the Beneficial Role of PPAR Agonists in Cardiac Diseases

Cardiac metabolic pathways affected in the mouse model of barth syndrome

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

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

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

How many minors are participating in clinical research today? An estimate and important lessons learned

A Zebrafish Model of Human Barth Syndrome Reveals the Essential Role of Tafazzin in Cardiac Development and Function

A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women

Evaluation of Fellows' CrossTalk Effectiveness in Promoting Transdisciplinary Networking and Research

Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases

Enhancing Pediatric Fellows' Research Training: Development of an Office of Pediatric Clinical Fellowships

Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening

Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry

Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein

Mechanism of Hyperinsulinism in Short-chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency Involves Activation of Glutamate Dehydrogenase

Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death

Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice

Cardiac-specific VLCAD deficiency induces dilated cardiomyopathy and cold intolerance

Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice

Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card

Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome : Impact of expanded newborn screening on their diagnosis and management

MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency

Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to β-subunit mutations

Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease

Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation

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