Metabolic pathways at the crossroads of diabetes and inborn errors

Lysine desuccinylase SIRT5 binds to cardiolipin and regulates the electron transport chain

(2017) Yuxun Zhang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Acute dietary fat intake initiates alterations in energy metabolism and insulin resistance

(2017) Elisa Álvarez Hernández et al.   JOURNAL OF CLINICAL INVESTIGATION

Loss of sirtuin 4 leads to elevated glucose- and leucine-stimulated insulin levels and accelerated age-induced insulin resistance in multiple murine genetic backgrounds

(2017) Frank K. Huynh et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Investigating the link of ACAD10 deficiency to type 2 diabetes mellitus

(2017) Kaitlyn Bloom et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Astrocyte IKKβ/NF-κB signaling is required for diet-induced obesity and hypothalamic inflammation

(2017) J.D. Douglass et al.   Molecular Metabolism

Pharmacological Inhibition of c-Jun N-terminal Kinase Reduces Food Intake and Sensitizes Leptin’s Anorectic Signaling Actions

(2017) Su Gao et al.   Scientific Reports

PLIN2 is a Key Regulator of the Unfolded Protein Response and Endoplasmic Reticulum Stress Resolution in Pancreatic β Cells

(2017) Elaine Chen et al.   Scientific Reports

Ketoisocaproic acid, a metabolite of leucine, suppresses insulin-stimulated glucose transport in skeletal muscle cells in a BCAT2-dependent manner

(2016) Mahshid Moghei et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Assessment of plasma acylcarnitines before and after weight loss in obese subjects

(2016) Marieke G. Schooneman et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Plasma acylcarnitines during insulin stimulation in humans are reflective of age-related metabolic dysfunction

(2016) Leslie A. Consitt et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Mitochondrial response to the BCKDK-deficiency: Some clues to understand the positive dietary response in this form of autism

(2016) A. Oyarzabal et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Catabolic Defect of Branched-Chain Amino Acids Promotes Heart FailureCLINICAL PERSPECTIVE

(2016) Haipeng Sun et al.   CIRCULATION

Biomarkers of Morbid Obesity and Prediabetes by Metabolomic Profiling of Human Discordant Phenotypes

(2016) Sara Tulipani et al.   CLINICA CHIMICA ACTA

The Hypoglycemic Phenotype Is Islet Cell–Autonomous in Short-Chain Hydroxyacyl-CoA Dehydrogenase–Deficient Mice

(2016) Anders Molven et al.   DIABETES

Branched-Chain Amino Acids and Insulin Metabolism: The Insulin Resistance Atherosclerosis Study (IRAS)

(2016) C. Christine Lee et al.   DIABETES CARE

Liver and Muscle Contribute Differently to the Plasma Acylcarnitine Pool During Fasting and Exercise in Humans

(2016) G. Xu et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Sirtuin activation as a therapeutic approach against inborn errors of metabolism

(2016) Jeannette C. Bleeker et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Isolated methylmalonic acidemia with unusual presentation mimicking diabetic ketoacidosis

(2016) Prapai Dejkhamron et al.   JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM

The genetic architecture of type 2 diabetes

(2016) Christian Fuchsberger et al.   NATURE

A branched-chain amino acid metabolite drives vascular fatty acid transport and causes insulin resistance

(2016) Cholsoon Jang et al.   NATURE MEDICINE

Slowing ageing by design: the rise of NAD+ and sirtuin-activating compounds

(2016) Michael S. Bonkowski et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Branched-chain and aromatic amino acids, insulin resistance and liver specific ectopic fat storage in overweight to obese subjects

(2016) S. Haufe et al.   NUTRITION METABOLISM AND CARDIOVASCULAR DISEASES

Decreased acylcarnitine content improves insulin sensitivity in experimental mice models of insulin resistance

(2016) Edgars Liepinsh et al.   PHARMACOLOGICAL RESEARCH

Suppression of Endogenous Glucose Production by Isoleucine and Valine and Impact of Diet Composition

(2016) Isabel Arrieta-Cruz et al.   Nutrients

Lipids Reprogram Metabolism to Become a Major Carbon Source for Histone Acetylation

(2016) Eoin McDonnell et al.   Cell Reports

Defects in muscle branched-chain amino acid oxidation contribute to impaired lipid metabolism

(2016) Carles Lerin et al.   Molecular Metabolism

Branched short-chain fatty acids modulate glucose and lipid metabolism in primary adipocytes

(2016) Emilia Heimann et al.   Adipocyte

Urea cycle regulation by mitochondrial sirtuin, SIRT5

(2016) Takashi Nakagawa et al.   Aging-US

SIRT3 Deficiency Induces Endothelial Insulin Resistance and Blunts Endothelial-Dependent Vasorelaxation in Mice and Human with Obesity

(2016) Lu Yang et al.   Scientific Reports

Mitochondrial fat oxidation is essential for lipid-induced inflammation in skeletal muscle in mice

(2016) Jaycob D. Warfel et al.   Scientific Reports

Resveratrol supplementation: Where are we now and where should we go?

(2015) Marta G. Novelle et al.   AGEING RESEARCH REVIEWS

Leucine partially protects muscle mass and function during bed rest in middle-aged adults1,2

(2015) Kirk L English et al.   AMERICAN JOURNAL OF CLINICAL NUTRITION

Long-chain acylcarnitines activate cell stress and myokine release in C2C12 myotubes: calcium-dependent and -independent effects

(2015) Colin S. McCoin et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

SIRT3 Is Crucial for Maintaining Skeletal Muscle Insulin Action and Protects Against Severe Insulin Resistance in High-Fat–Fed Mice

(2015) Louise Lantier et al.   DIABETES

Acylcarnitines: potential implications for skeletal muscle insulin resistance

(2015) Céline Aguer et al.   FASEB JOURNAL

Quo vadis: the re-definition of “inborn metabolic diseases”

(2015) Eva Morava et al.   JOURNAL OF INHERITED METABOLIC DISEASE

The role of fatty acids in insulin resistance

(2015) Barry Sears et al.   Lipids in Health and Disease

Hypothalamic innate immune reaction in obesity

(2015) Stefanie Kälin et al.   Nature Reviews Endocrinology

Acylcarnitines—old actors auditioning for new roles in metabolic physiology

(2015) Colin S. McCoin et al.   Nature Reviews Endocrinology

Leptin resistance in diet-induced obesity: the role of hypothalamic inflammation

(2015) K. C. G. de Git et al.   Obesity Reviews

Impaired mitochondrial fat oxidation induces adaptive remodeling of muscle metabolism

(2015) Shawna E. Wicks et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Genetics of Type 2 Diabetes—Pitfalls and Possibilities

(2015) Rashmi Prasad et al.   Genes

SIRT3 and SIRT5 Regulate the Enzyme Activity and Cardiolipin Binding of Very Long-Chain Acyl-CoA Dehydrogenase

(2015) Yuxun Zhang et al.   PLoS One

Acylcarnitines activate proinflammatory signaling pathways

(2014) Jennifer M. Rutkowsky et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Plasma acylcarnitines inadequately reflect tissue acylcarnitine metabolism

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

Lysine Glutarylation Is a Protein Posttranslational Modification Regulated by SIRT5

(2014) Minjia Tan et al.   Cell Metabolism

NAD+-Dependent Activation of Sirt1 Corrects the Phenotype in a Mouse Model of Mitochondrial Disease

(2014) Raffaele Cerutti et al.   Cell Metabolism

Lipotoxicity in the Pancreatic Beta Cell: Not Just Survival and Function, but Proliferation as Well?

(2014) Rohit B. Sharma et al.   Current Diabetes Reports

Mitochondrial protein acetylation is driven by acetyl-CoA from fatty acid oxidation

(2014) Olga Pougovkina et al.   HUMAN MOLECULAR GENETICS

Aberrant protein acylation is a common observation in inborn errors of acyl-CoA metabolism

(2014) Olga Pougovkina et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Nonenzymatic Protein Acylation as a Carbon Stress Regulated by Sirtuin Deacylases

(2014) Gregory R. Wagner et al.   MOLECULAR CELL

Resveratrol treatment as an adjunct to pharmacological management in type 2 diabetes mellitus-systematic review and meta-analysis

(2014) Heather A. Hausenblas et al.   MOLECULAR NUTRITION & FOOD RESEARCH

Evidence That Multiple Defects in Lipid Regulation Occur before Hyperglycemia during the Prodrome of Type-2 Diabetes

(2014) Simon G. Anderson et al.   PLoS One

Altered body composition and energy expenditure but normal glucose tolerance among humans with a long-chain fatty acid oxidation disorder

(2013) Melanie B. Gillingham et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

SIRT5 Regulates the Mitochondrial Lysine Succinylome and Metabolic Networks

(2013) Matthew J. Rardin et al.   Cell Metabolism

Hypothalamic Inflammation: Marker or Mechanism of Obesity Pathogenesis?

(2013) Joshua P. Thaler et al.   DIABETES

Shared genetic influence of BMI, physical activity and type 2 diabetes: a twin study

(2013) S. Carlsson et al.   DIABETOLOGIA

Carnitine palmitoyltransferase 2 and carnitine/acylcarnitine translocase are involved in the mitochondrial synthesis and export of acylcarnitines

(2013) Sara Violante et al.   FASEB JOURNAL

Selective hepatic insulin resistance in a murine model heterozygous for a mitochondrial trifunctional protein defect

(2013) R. Scott Rector et al.   HEPATOLOGY

Acute Inhibition of Central c-Jun N-terminal Kinase Restores Hypothalamic Insulin Signalling and Alleviates Glucose Intolerance in Diabetic Mice

(2013) J. Benzler et al.   JOURNAL OF NEUROENDOCRINOLOGY

SIRT4 Represses Peroxisome Proliferator-Activated Receptor α Activity To Suppress Hepatic Fat Oxidation

(2013) Gaëlle Laurent et al.   MOLECULAR AND CELLULAR BIOLOGY

SIRT4 Coordinates the Balance between Lipid Synthesis and Catabolism by Repressing Malonyl CoA Decarboxylase

(2013) Gaëlle Laurent et al.   MOLECULAR CELL

Leucine Supplementation Protects from Insulin Resistance by Regulating Adiposity Levels

(2013) Elke Binder et al.   PLoS One

Circadian Clock NAD+ Cycle Drives Mitochondrial Oxidative Metabolism in Mice

(2013) C. B. Peek et al.   SCIENCE

TOR-centric view on insulin resistance and diabetic complications: perspective for endocrinologists and gerontologists

(2013) M V Blagosklonny   Cell Death & Disease

Lipid Sensing and Insulin Resistance in the Brain

(2012) Jessica T.Y. Yue et al.   Cell Metabolism

Inhibition of Hypothalamic Inflammation Reverses Diet-Induced Insulin Resistance in the Liver

(2012) M. Milanski et al.   DIABETES

Novel Insights Into Fatty Acid Oxidation, Amino Acid Metabolism, and Insulin Secretion From Studying Patients With Loss of Function Mutations in 3-Hydroxyacyl-CoA Dehydrogenase

(2012) Amanda J. Heslegrave et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Augmenting muscle diacylglycerol and triacylglycerol content by blocking fatty acid oxidation does not impede insulin sensitivity

(2012) S. Timmers et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Obesity is associated with hypothalamic injury in rodents and humans

(2011) Joshua P. Thaler et al.   JOURNAL OF CLINICAL INVESTIGATION

SIRT3 Deficiency and Mitochondrial Protein Hyperacetylation Accelerate the Development of the Metabolic Syndrome

(2011) Matthew D. Hirschey et al.   MOLECULAR CELL

Methylmalonic acidemia mimicking diabetic ketoacidosis in an infant

(2011) A Guven et al.   PEDIATRIC DIABETES

Dietary Leucine - An Environmental Modifier of Insulin Resistance Acting on Multiple Levels of Metabolism

(2011) Yazmin Macotela et al.   PLoS One

Sirtuin-3 (Sirt3) regulates skeletal muscle metabolism and insulin signaling via altered mitochondrial oxidation and reactive oxygen species production

(2011) E. Jing et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Resistance to High-Fat Diet-Induced Obesity and Insulin Resistance in Mice with Very Long-Chain Acyl-CoA Dehydrogenase Deficiency

(2010) Dongyan Zhang et al.   Cell Metabolism

Branched-Chain Amino Acid Supplementation Promotes Survival and Supports Cardiac and Skeletal Muscle Mitochondrial Biogenesis in Middle-Aged Mice

(2010) Giuseppe D'Antona et al.   Cell Metabolism

Variants in ACAD10 are associated with type 2 diabetes, insulin resistance and lipid oxidation in Pima Indians

(2010) L. Bian et al.   DIABETOLOGIA

Evidence that BMI and type 2 diabetes share only a minor fraction of genetic variance: a follow-up study of 23,585 monozygotic and dizygotic twins from the Finnish Twin Cohort Study

(2010) M. Lehtovirta et al.   DIABETOLOGIA

Role of the hypothalamic-pituitary-thyroid axis in metabolic regulation by JNK1

(2010) G. Sabio et al.   GENES & DEVELOPMENT

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

(2010) Changhong Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Identification and characterization of new long chain Acyl-CoA dehydrogenases

(2010) Miao He et al.   MOLECULAR GENETICS AND METABOLISM

Plasma Metabolomic Profiles Reflective of Glucose Homeostasis in Non-Diabetic and Type 2 Diabetic Obese African-American Women

(2010) Oliver Fiehn et al.   PLoS One

Hypothalamic and pituitary c-Jun N-terminal kinase 1 signaling coordinately regulates glucose metabolism

(2010) B. F. Belgardt et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Branched-Chain Amino Acid-Related Metabolic Signature that Differentiates Obese and Lean Humans and Contributes to Insulin Resistance

(2009) Christopher B. Newgard et al.   Cell Metabolism

Protein phosphatase 2Cm is a critical regulator of branched-chain amino acid catabolism in mice and cultured cells

(2009) Gang Lu et al.   JOURNAL OF CLINICAL INVESTIGATION

Animal models of maple syrup urine disease

(2009) K. J. Skvorak   JOURNAL OF INHERITED METABOLIC DISEASE

Saturated Fatty Acids Produce an Inflammatory Response Predominantly through the Activation of TLR4 Signaling in Hypothalamus: Implications for the Pathogenesis of Obesity

(2009) M. Milanski et al.   JOURNAL OF NEUROSCIENCE

Plasma Acylcarnitine Profiles Suggest Incomplete Long-Chain Fatty Acid β-Oxidation and Altered Tricarboxylic Acid Cycle Activity in Type 2 Diabetic African-American Women

(2009) Sean H. Adams et al.   JOURNAL OF NUTRITION

From chronic overnutrition to insulin resistance: The role of fat-storing capacity and inflammation

(2009) L. Lionetti et al.   NUTRITION METABOLISM AND CARDIOVASCULAR DISEASES

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

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

Muscle acylcarnitines during short-term fasting in lean healthy men

(2008) Maarten R. Soeters et al.   CLINICAL SCIENCE

Overexpression of Carnitine Palmitoyltransferase-1 in Skeletal Muscle Is Sufficient to Enhance Fatty Acid Oxidation and Improve High-Fat Diet-Induced Insulin Resistance

(2008) C. R. Bruce et al.   DIABETES