Triheptanoin: long-term effects in the very long-chain acyl-CoA dehydrogenase-deficient mouse

Triheptanoin treatment in patients with pediatric cardiomyopathy associated with long chain-fatty acid oxidation disorders

(2016) J. Vockley et al.   MOLECULAR GENETICS AND METABOLISM

Sexual dimorphism of lipid metabolism in very long-chain acyl-CoA dehydrogenase deficient (VLCAD−/−) mice in response to medium-chain triglycerides (MCT)

(2015) Sara Tucci et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

De novofatty acid biosynthesis and elongation in very long-chain acyl-CoA dehydrogenase-deficient mice supplemented with odd or even medium-chain fatty acids

(2015) Sara Tucci et al.   FEBS Journal

Triheptanoin Alleviates Ventricular Hypertrophy and Improves Myocardial Glucose Oxidation in Rats With Pressure Overload

(2015) T. Dung Nguyen et al.   JOURNAL OF CARDIAC FAILURE

Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment—A retrospective chart review

(2015) Jerry Vockley et al.   MOLECULAR GENETICS AND METABOLISM

Triheptanoin improves brain energy metabolism in patients with Huntington disease

(2015) I. M. Adanyeguh et al.   NEUROLOGY

Reduced severity of ischemic stroke and improvement of mitochondrial function after dietary treatment with the anaplerotic substance triheptanoin

(2015) T.M. Schwarzkopf et al.   NEUROSCIENCE

Development and pathomechanisms of cardiomyopathy in very long-chain acyl-CoA dehydrogenase deficient (VLCAD−/−) mice

(2014) Sara Tucci et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Inborn errors of metabolism identified via newborn screening: Ten-year incidence data and costs of nutritional interventions for research agenda planning

(2014) Bradford L. Therrell et al.   MOLECULAR GENETICS AND METABOLISM

Triheptanoin for Glucose Transporter Type I Deficiency (G1D)

(2014) Juan M. Pascual et al.   JAMA Neurology

Reprint of “Glutathione Peroxidase Activity in Selenium-Deficient Rat Liver”

(2012) Richard A. Lawrence   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Triheptanoin in acute mouse seizure models

(2012) Nicola K. Thomas et al.   EPILEPSY RESEARCH

Transcriptional Regulation of the Fetal Cardiac Gene Program

(2012) Koichiro Kuwahara et al.   JOURNAL OF PHARMACOLOGICAL SCIENCES

Tissue-Specific Strategies of the Very-Long Chain Acyl-CoA Dehydrogenase-Deficient (VLCAD−/−) Mouse to Compensate a Defective Fatty Acid β-Oxidation

(2012) Sara Tucci et al.   PLoS One

Disrupted fat distribution and composition due to medium-chain triglycerides in mice with a β-oxidation defect

(2011) Sara Tucci et al.   AMERICAN JOURNAL OF CLINICAL NUTRITION

Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's disease

(2010) Fanny Mochel et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Fasting-induced oxidative stress in very long chain acyl-CoA dehydrogenase-deficient mice

(2010) Sara Tucci et al.   FEBS Journal

Pre-exercise medium-chain triglyceride application prevents acylcarnitine accumulation in skeletal muscle from very-long-chain acyl-CoA-dehydrogenase-deficient mice

(2010) Sonja Primassin et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Newborn screening for disorders of fatty-acid oxidation: experience and recommendations from an expert meeting

(2010) Martin Lindner et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Current issues regarding treatment of mitochondrial fatty acid oxidation disorders

(2010) Ute Spiekerkoetter et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Medium-chain triglycerides impair lipid metabolism and induce hepatic steatosis in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient mice

(2010) Sara Tucci et al.   MOLECULAR GENETICS AND METABOLISM

Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profile

(2009) Lei Gu et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Treatment recommendations in long-chain fatty acid oxidation defects: consensus from a workshop

(2009) U. Spiekerkoetter et al.   JOURNAL OF INHERITED METABOLIC DISEASE

A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency

(2009) Georgianne L. Arnold et al.   MOLECULAR GENETICS AND METABOLISM

Carnitine palmitoyltransferase II deficiency: Successful anaplerotic diet therapy

(2008) C. R. Roe et al.   NEUROLOGY

Comparison between surrogate indexes of insulin sensitivity and resistance and hyperinsulinemic euglycemic clamp estimates in mice

(2007) Sihoon Lee et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM