All Stories

  1. Vitamin K as an add-on therapy in pyridoxine-dependent epilepsy
  2. Odd-chain dicarboxylic acid feeding recapitulates the biochemical phenotype of glutaric aciduria type 1 in mice
  3. A case of hyperlysinemia identified by urine newborn screening
  4. Acyl‐CoA dehydrogenase substrate promiscuity: Challenges and opportunities for development of substrate reduction therapy in disorders of valine and isoleucine metabolism
  5. The biochemistry and physiology of long-chain dicarboxylic acid metabolism
  6. A temporal classifier predicts histopathology state and parses acute-chronic phasing in inflammatory bowel disease patients
  7. Acyl-CoA dehydrogenase substrate promiscuity limits the potential for development of substrate reduction therapy in disorders of valine and isoleucine metabolism
  8. Characterization and structure of the human lysine-2-oxoglutarate reductase domain, a novel therapeutic target for treatment of glutaric aciduria type 1
  9. Functional Versatility of the Human 2-Oxoadipate Dehydrogenase in the L-Lysine Degradation Pathway toward Its Non-Cognate Substrate 2-Oxopimelic Acid
  10. Characterization and structure of the human lysine-2-oxoglutarate reductase domain, a novel therapeutic target for treatment of glutaric aciduria type 1
  11. The peroxisomal transporter ABCD3 plays a major role in hepatic dicarboxylic fatty acid metabolism and lipid homeostasis
  12. Peroxisomal L-bifunctional Protein Deficiency Causes Male-specific Kidney Hypertrophy and Proximal Tubular Injury in Mice
  13. The peroxisomal transporter ABCD3 plays a major role in dicarboxylic fatty acid metabolism
  14. Murine deficiency of peroxisomal l-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol homeostasis
  15. A mitochondrial long-chain fatty acid oxidation defect leads to uncharged tRNA accumulation and activation of the integrated stress response in the mouse heart
  16. Deficiency of peroxisomal L-bifunctional protein (EHHADH) causes male-specific kidney hypertrophy and proximal tubular injury in mice
  17. Murine deficiency of peroxisomal L-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol homeostasis
  18. Dietary restriction in the long-chain acyl-CoA dehydrogenase knockout mouse
  19. Glutaric aciduria type 3 is a naturally occurring biochemical trait in inbred mice of 129 substrains
  20. Deletion of 2‐aminoadipic semialdehyde synthase limits metabolite accumulation in cell and mouse models for glutaric aciduria type 1
  21. Structure–function analyses of the G729R 2-oxoadipate dehydrogenase genetic variant associated with a disorder of l-lysine metabolism
  22. Slc22a5 haploinsufficiency does not aggravate the phenotype of the long‐chain acyl‐CoA dehydrogenase KO mouse
  23. DHTKD1 and OGDH display in vivo substrate overlap and form a hybrid ketoacid dehydrogenase complex
  24. Saccharopine, a lysine degradation intermediate, is a mitochondrial toxin
  25. Metabolic crosstalk between organelles
  26. News and views
  27. Deficiency of the Mitochondrial NAD Kinase Causes Stress-Induced Hepatic Steatosis in Mice
  28. Changes in the Metabolome in Response to Low-Dose Exposure to Environmental Chemicals Used in Personal Care Products during Different Windows of Susceptibility
  29. In vivomouse myocardial31P MRS using three-dimensional image-selectedin vivospectroscopy (3D ISIS): technical considerations and biochemical validations
  30. Transorgan fluxes in a porcine model reveal a central role for liver in acylcarnitine metabolism
  31. Corrigendum
  32. Novel, Compound Heterozygous, Single-Nucleotide Variants inMARS2Associated with Developmental Delay, Poor Growth, and Sensorineural Hearing Loss
  33. Fiber-type-specific sensitivities and phenotypic adaptations to dietary fat overload differentially impact fast- versus slow-twitch muscle contractile function in C57BL/6J mice
  34. Cholesterol-Induced Hepatic Inflammation Does Not Underlie the Predisposition to Insulin Resistance in Dyslipidemic Female LDL Receptor Knockout Mice
  35. High-protein diets prevent steatosis and induce hepatic accumulation of monomethyl branched-chain fatty acids
  36. Pioglitazone treatment restoresin vivomuscle oxidative capacity in a rat model of diabetes
  37. SUMOylation-Dependent LRH-1/PROX1 Interaction Promotes Atherosclerosis by Decreasing Hepatic Reverse Cholesterol Transport
  38. Bile acids alter male fertility through G-protein-coupled bile acid receptor 1 signaling pathways in mice
  39. Multilayered Genetic and Omics Dissection of Mitochondrial Activity in a Mouse Reference Population
  40. A Mitochondrial Expatriate: Nuclear Pyruvate Dehydrogenase
  41. Plasma acylcarnitines inadequately reflect tissue acylcarnitine metabolism
  42. In vivo proton T1relaxation times of mouse myocardial metabolites at 9.4 T
  43. Mitochondrial NADP(H) deficiency due to a mutation in NADK2 causes dienoyl-CoA reductase deficiency with hyperlysinemia
  44. Fasting Serum Taurine-Conjugated Bile Acids Are Elevated in Type 2 Diabetes and Do Not Change With Intensification of Insulin
  45. Optimizing anesthetic regimen for surgery in mice through minimization of hemodynamic, metabolic, and inflammatory perturbations
  46. Food withdrawal lowers energy expenditure and induces inactivity in long-chain fatty acid oxidation-deficient mouse models
  47. Aberrant protein acylation is a common observation in inborn errors of acyl-CoA metabolism
  48. Mitochondrial protein acetylation is driven by acetyl-CoA from fatty acid oxidation
  49. Identification and characterization of Eci3, a murine kidney-specific  3, 2-enoyl-CoA isomerase
  50. Muscle MRI in patients with long-chain fatty acid oxidation disorders
  51. ACAD9, a complex I assembly factor with a moonlighting function in fatty acid oxidation deficiencies
  52. Myocardial energy shortage and unmet anaplerotic needs in the fasted long-chain acyl-CoA dehydrogenase knockout mouse
  53. Peroxisomes contribute to the acylcarnitine production when the carnitine shuttle is deficient
  54. Biochemical Competition Makes Fatty-Acid β-Oxidation Vulnerable to Substrate Overload
  55. Impaired amino acid metabolism contributes to fasting-induced hypoglycemia in fatty acid oxidation defects
  56. Substrate specificity of human carnitine acetyltransferase: Implications for fatty acid and branched-chain amino acid metabolism
  57. Prevention and reversal of hepatic steatosis with a high-protein diet in mice
  58. Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice
  59. Overexpression of PLIN5 in skeletal muscle promotes oxidative gene expression and intramyocellular lipid content without compromising insulin sensitivity
  60. Carnitine palmitoyltransferase 2 and carnitine/acylcarnitine translocase are involved in the mitochondrial synthesis and export of acylcarnitines
  61. Genetic basis of hyperlysinemia
  62. Acylcarnitines
  63. Adaptive reciprocity of lipid and glucose metabolism in human short-term starvation
  64. Bile Acid Binding Resin Improves Metabolic Control through the Induction of Energy Expenditure
  65. Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids
  66. Characterization of D-3-hydroxybutyrylcarnitine (ketocarnitine): an identified ketosis-induced metabolite
  67. Improving the description of metabolic networks: the TCA cycle as example
  68. Muscle or liver-specific Sirt3 deficiency induces hyperacetylation of mitochondrial proteins without affecting global metabolic homeostasis
  69. A Diet Rich in Unsaturated Fatty Acids Prevents Progression Toward Heart Failure in a Rabbit Model of Pressure and Volume Overload
  70. Peroxisomal L-bifunctional enzyme (Ehhadh) is essential for the production of medium-chain dicarboxylic acids
  71. Bezafibrate lowers very long-chain fatty acids in X-linked adrenoleukodystrophy fibroblasts by inhibiting fatty acid elongation
  72. Treatment of genetically obese mice with the iminosugar N-(5-adamantane-1-yl-methoxy-pentyl)-deoxynojirimycin reduces body weight by decreasing food intake and increasing fat oxidation
  73. Role of Medium- and Short-Chain L-3-Hydroxyacyl-CoA Dehydrogenase in the Regulation of Body Weight and Thermogenesis
  74. New Driver for Lipid Synthesis
  75. The metabolic footprint of aging in mice
  76. Fasting-Induced Myocardial Lipid Accumulation in Long-Chain Acyl-CoA Dehydrogenase Knockout Mice Is Accompanied by Impaired Left Ventricular Function
  77. Differential effects of short- and long-term high-fat diet feeding on hepatic fatty acid metabolism in rats
  78. Lowering Bile Acid Pool Size with a Synthetic Farnesoid X Receptor (FXR) Agonist Induces Obesity and Diabetes through Reduced Energy Expenditure
  79. Post-natal myogenic and adipogenic developmental
  80. PARP-2 Regulates SIRT1 Expression and Whole-Body Energy Expenditure
  81. Critical assessment of human metabolic pathway databases: a stepping stone for future integration
  82. Acyl-CoA Dehydrogenase 9 Is Required for the Biogenesis of Oxidative Phosphorylation Complex I
  83. The Effects of Long- or Medium-Chain Fat Diets on Glucose Tolerance and Myocellular Content of Lipid Intermediates in Rats
  84. The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results
  85. A general introduction to the biochemistry of mitochondrial fatty acid β-oxidation
  86. Effect of Statins on the Viability of Macrophages and Smooth Muscle Cells
  87. Glutamine Synthetase in Muscle Is Required for Glutamine Production during Fasting and Extrahepatic Ammonia Detoxification
  88. Increased mitochondrial content rescues in vivo muscle oxidative capacity in long-term high-fat-diet-fed rats
  89. Mitochondrial long chain fatty acid β-oxidation in man and mouse
  90. Serum Bile Acids Are Higher in Humans With Prior Gastric Bypass: Potential Contribution to Improved Glucose and Lipid Metabolism
  91. Muscle acylcarnitines during short-term fasting in lean healthy men
  92. Effects of Insulin on Ketogenesis Following Fasting in Lean and Obese Men
  93. Pyruvate dehydrogenase kinase 4 expression is synergistically induced by AMP-activated protein kinase and fatty acids
  94. Specific SIRT1 Activation Mimics Low Energy Levels and Protects against Diet-Induced Metabolic Disorders by Enhancing Fat Oxidation
  95. A key role for the peroxisomal ABCD2 transporter in fatty acid homeostasis
  96. Metabolomics: Unraveling the chemical individuality of common human diseases
  97. Specific SIRT1 Activation Mimics Low Energy Levels and Protects against Diet-Induced Metabolic Disorders by Enhancing Fat Oxidation
  98. Increased intramyocellular lipid content but normal skeletal muscle mitochondrial oxidative capacity throughout the pathogenesis of type 2 diabetes
  99. The Glucosylceramide Synthase InhibitorN-(5-Adamantane-1-yl-methoxy-pentyl)-deoxynojirimycin Induces Sterol Regulatory Element-Binding Protein-Regulated Gene Expression and Cholesterol Synthesis in HepG2 Cells
  100. A cholesterol-free, high-fat diet suppresses gene expression of cholesterol transporters in murine small intestine
  101. Characterization of l-aminocarnitine, an inhibitor of fatty acid oxidation
  102. Peroxisome Proliferator-activated Receptor (PPAR)-2 Controls Adipocyte Differentiation and Adipose Tissue Function through the Regulation of the Activity of the Retinoid X Receptor/PPARγ Heterodimer
  103. Compromised Intestinal Lipid Absorption in Mice with a Liver-Specific Deficiency of Liver Receptor Homolog 1
  104. In VivoImaging of Farnesoid X Receptor Activity Reveals the Ileum as the Primary Bile Acid Signaling Tissue
  105. A liver revival featuring bile acids
  106. The small heterodimer partner is a gonadal gatekeeper of sexual maturation in male mice
  107. Homing in on bile acid physiology
  108. Peroxisomes and bile acid biosynthesis
  109. Endocrine functions of bile acids
  110. Bile acids induce energy expenditure by promoting intracellular thyroid hormone activation
  111. Manipulation of isoprenoid biosynthesis as a possible therapeutic option in mevalonate kinase deficiency
  112. PGC-1α
  113. Bile acids lower triglyceride levels via a pathway involving FXR, SHP, and SREBP-1c
  114. Peroxisome proliferator-activated receptor-γ: too much of a good thing causes harm
  115. The enterohepatic nuclear receptors are major regulators of the enterohepatic circulation of bile salts
  116. Compensation by the muscle limits the metabolic consequences of lipodystrophy in PPARγ hypomorphic mice
  117. Carrier frequency of the V377I (1129G>A) MVK mutation, associated with Hyper-IgD and periodic fever syndrome, in the Netherlands
  118. Regulation of Isoprenoid/Cholesterol Biosynthesis in Cells from Mevalonate Kinase-deficient Patients
  119. Hyper IgD syndrome (HIDS) associated with in vitro evidence of defective monocyte TNFRSF1A shedding and partial response to TNF receptor blockade with etanercept
  120. Lack of isoprenoid products raises ex vivo interleukin-1? secretion in hyperimmunoglobulinemia D and periodic fever syndrome
  121. Erratum: Organization of the mevalonate kinase (MVK) gene and identification of novel mutations causing mevalonic aciduria and hyperimmunoglobulinaemia D and periodic fever syndrome
  122. Organization of the mevalonate kinase (MVK) gene and identification of novel mutations causing mevalonic aciduria and hyperimmunoglobulinaemia D and periodic fever syndrome
  123. Nonorthologous Gene Displacement of Phosphomevalonate Kinase
  124. Biochemical and genetic aspects of mevalonate kinase and its deficiency
  125. Identification and Characterization of Three Novel Missense Mutations in Mevalonate Kinase cDNA Causing Mevalonic Aciduria, a Disorder of Isoprene Biosynthesis
  126. Mutations in MVK, encoding mevalonate kinase, cause hyperimmunoglobulinaemiaD and periodic fever syndrome