The promiscuous enzyme medium-chain 3-keto-acyl-CoA thiolase triggers a vicious cycle in fatty-acid beta-oxidation

The Biochemistry and Physiology of Mitochondrial Fatty Acid β-Oxidation and Its Genetic Disorders

(2016) Sander M. Houten et al.   Annual Review of Physiology

Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders

(2016) Karen van Eunen et al.   BMC BIOLOGY

p46Shc Inhibits Thiolase and Lipid Oxidation in Mitochondria

(2016) Alexey Tomilov et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutation of the mitochondrial carrier SLC25A42 causes a novel form of mitochondrial myopathy in humans

(2015) Hanan E. Shamseldin et al.   HUMAN GENETICS

Extracellular 4′-phosphopantetheine is a source for intracellular coenzyme A synthesis

(2015) Balaji Srinivasan et al.   Nature Chemical Biology

Metabolic and Tissue-Specific Regulation of Acyl-CoA Metabolism

(2015) Jessica M. Ellis et al.   PLoS One

Plasma acylcarnitines inadequately reflect tissue acylcarnitine metabolism

(2014) Marieke G. Schooneman et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Metabolic Inflexibility: When Mitochondrial Indecision Leads to Metabolic Gridlock

(2014) Deborah M. Muoio   CELL

Acyl-CoA thioesterase-2 facilitates mitochondrial fatty acid oxidation in the liver

(2014) Cynthia Moffat et al.   JOURNAL OF LIPID RESEARCH

Acyl-CoA thioesterase 9 (ACOT9) in mouse may provide a novel link between fatty acid and amino acid metabolism in mitochondria

(2013) Veronika Tillander et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice

(2013) Adrianus J. Bakermans et al.   JOURNAL OF INHERITED METABOLIC DISEASE

In vitro and in vivo consequences of variant medium-chain acyl-CoA dehydrogenase genotypes

(2013) Catharina ML Touw et al.   Orphanet Journal of Rare Diseases

Biochemical Competition Makes Fatty-Acid β-Oxidation Vulnerable to Substrate Overload

(2013) Karen van Eunen et al.   PLoS Computational Biology

Risk stratification by residual enzyme activity after newborn screening for medium-chain acyl-CoA dehyrogenase deficiency: data from a cohort study

(2012) Catharina M L Touw et al.   Orphanet Journal of Rare Diseases

Short-Chain 3-Hydroxyacyl-Coenzyme A Dehydrogenase Associates with a Protein Super-Complex Integrating Multiple Metabolic Pathways

(2012) Srinivas B. Narayan et al.   PLoS One

Evidence for Physical Association of Mitochondrial Fatty Acid Oxidation and Oxidative Phosphorylation Complexes

(2010) Yudong Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A general introduction to the biochemistry of mitochondrial fatty acid β-oxidation

(2010) Sander Michel Houten 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

A Novel Member of Solute Carrier Family 25 (SLC25A42) Is a Transporter of Coenzyme A and Adenosine 3′,5′-Diphosphate in Human Mitochondria

(2009) Giuseppe Fiermonte et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial Overload and Incomplete Fatty Acid Oxidation Contribute to Skeletal Muscle Insulin Resistance

(2008) Timothy R. Koves et al.   Cell Metabolism

Neonatal screening for medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in The Netherlands: The importance of enzyme analysis to ascertain true MCAD deficiency

(2008) T. G. J. Derks et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Hereditary and acquired diseases of acyl-coenzyme A metabolism

(2008) Grant A Mitchell et al.   MOLECULAR GENETICS AND METABOLISM