Very long-chain acyl-CoA dehydrogenase (VLCAD-) deficiency–studies on treatment effects and long-term outcomes in mouse models

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

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

VLCAD deficiency: Follow-up and outcome of patients diagnosed through newborn screening in Victoria

(2016) Maureen Evans 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

Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15years Experience

(2015) Charles R. Roe et al.   MOLECULAR GENETICS AND METABOLISM

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

Long-chain Acyl-CoA Dehydrogenase Deficiency as a Cause of Pulmonary Surfactant Dysfunction

(2014) Eric S. Goetzman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Plasma concentration of cis9trans11 CLA in males and females is influenced by SCD1 genetic variations and hormonal contraceptives: a cross-sectional study

(2013) Salma A Abdelmagid et al.   Nutrition & Metabolism

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

Fasting-Induced Myocardial Lipid Accumulation in Long-Chain Acyl-CoA Dehydrogenase Knockout Mice Is Accompanied by Impaired Left Ventricular Function

(2011) Adrianus J. Bakermans et al.   Circulation-Cardiovascular Imaging

Substrate oxidation and cardiac performance during exercise in disorders of long chain fatty acid oxidation

(2011) Annie M. Behrend et al.   MOLECULAR GENETICS AND METABOLISM

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

(2010) Sara Tucci et al.   FEBS Journal

Peroxisomal and Microsomal Lipid Pathways Associated with Resistance to Hepatic Steatosis and Reduced Pro-inflammatory State

(2010) Diana Hall et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse models

(2010) Ute Spiekerkoetter 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

Low expression of long-chain acyl-CoA dehydrogenase in human skeletal muscle

(2010) Amy C. Maher et al.   MOLECULAR GENETICS AND METABOLISM

Mitochondrial long chain fatty acid β-oxidation in man and mouse

(2009) Malika Chegary et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Dysfunctional very-low-density lipoprotein synthesis and release is a key factor in nonalcoholic steatohepatitis pathogenesis

(2009) Koji Fujita et al.   HEPATOLOGY

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

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

Cardiac hypertrophy in mice with long-chain acyl-CoA dehydrogenase or very long-chain acyl-CoA dehydrogenase deficiency

(2009) Keith B Cox et al.   LABORATORY INVESTIGATION

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

Sex differences in obesity and the regulation of energy homeostasis

(2009) J. C. Lovejoy et al.   Obesity Reviews

Identification of a Lipokine, a Lipid Hormone Linking Adipose Tissue to Systemic Metabolism

(2008) Haiming Cao et al.   CELL