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Journal Article
Molecular & cellular proteomics, ISSN 1535-9476, 2015, Volume 14, Issue 11, pp. 3056 - 3071
Journal Article
Journal Article
Brain, ISSN 0006-8950, 8/2007, Volume 130, Issue 8, pp. 2045 - 2054
Multiple acyl-CoA dehydrogenation deficiency (MADD) is a disorder of fatty acid, amino acid and choline metabolism that can result from defects in two... 
Mutations | Riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency | Mitochondrial myopathy | Electron transfer flavoprotein ubiquinone oxidoreductase | riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency | ACYLCOENZYME-A DEHYDROGENASE | BETA-OXIDATION | OXIDATIVE-PHOSPHORYLATION | UBIQUINONE OXIDOREDUCTASE | ACIDEMIA TYPE-II | INTACT CULTURED FIBROBLASTS | ACIDURIA TYPE-II | NEUROSCIENCES | CLINICAL NEUROLOGY | LIPID-STORAGE MYOPATHY | SKELETAL-MUSCLE | mitochondrial myopathy | electron transfer flavoprotein ubiquinone | mutations | ELECTRON-TRANSFER FLAVOPROTEIN | oxidoreductase | Mitochondrial Myopathies - metabolism | Humans | Mitochondria, Muscle - metabolism | Child, Preschool | Iron-Sulfur Proteins - genetics | Male | Muscle, Skeletal - metabolism | Adult | Female | Carnitine - analogs & derivatives | Electron Transport - physiology | Brain Diseases, Metabolic - enzymology | Child | Fatty Acids - metabolism | Electron-Transferring Flavoproteins - genetics | Brain Diseases, Metabolic - genetics | Oxidation-Reduction | Riboflavin - therapeutic use | Carnitine - blood | Metabolism, Inborn Errors - metabolism | Oxidoreductases Acting on CH-NH Group Donors - genetics | Metabolism, Inborn Errors - genetics | Mitochondrial Myopathies - pathology | Mitochondrial Myopathies - drug therapy | Mitochondrial Myopathies - genetics | Acyl-CoA Dehydrogenase - deficiency | Adolescent | Muscle, Skeletal - pathology | Mutation | Metabolism, Inborn Errors - pathology | Index Medicus | Abridged Index Medicus | Electron Transport | Fatty Acids | pathology | Oxidoreductases Acting on CH-NH Group Donors | Riboflavin | Metabolic | MEDICIN OCH HÄLSOVETENSKAP | Acyl-CoA Dehydrogenase | Skeletal | Inborn Errors | Mitochondria | genetics | Brain Diseases | Muscle | MEDICAL AND HEALTH SCIENCES | drug therapy | Mitochondrial Myopathies | Electron-Transferring Flavoproteins | Preschool | physiology | blood | Iron-Sulfur Proteins | enzymology | Carnitine | deficiency | analogs & derivatives | metabolism | therapeutic use
Journal Article
Journal of Clinical Investigation, ISSN 0021-9738, 12/2014, Volume 124, Issue 12, pp. 5175 - 5190
Nonalcoholic fatty liver disease (NAFLD) spectrum disorders affect approximately 1 billion individuals worldwide. However, the drivers of progressive... 
HMG-COA SYNTHASE | MEDICINE, RESEARCH & EXPERIMENTAL | PPAR-ALPHA | PERFUSED RAT LIVER | HEPATIC INSULIN-RESISTANCE | MITOCHONDRIAL 3-HYDROXY-3-METHYLGLUTARYL-COA SYNTHASE | CARDIOVASCULAR-DISEASE | KETONE-BODY METABOLISM | ACID OXIDATION | COENZYME-A METABOLISM | MASS-SPECTROMETRY | Mitochondrial Diseases - pathology | Non-alcoholic Fatty Liver Disease - pathology | Gluconeogenesis - drug effects | Hydroxymethylglutaryl-CoA Synthase - genetics | Mitochondrial Diseases - metabolism | Acyl Coenzyme A - genetics | Male | Hydroxymethylglutaryl-CoA Synthase - metabolism | Hypoglycemia - pathology | Hyperglycemia - genetics | Hyperglycemia - chemically induced | Non-alcoholic Fatty Liver Disease - chemically induced | Mice, Mutant Strains | Hyperglycemia - pathology | Hydroxymethylglutaryl-CoA Synthase - deficiency | Acyl Coenzyme A - metabolism | Hypoglycemia - genetics | Mitochondrial Diseases - genetics | Dietary Fats - adverse effects | Non-alcoholic Fatty Liver Disease - genetics | Citric Acid Cycle - drug effects | Metabolism, Inborn Errors - metabolism | Glucose - genetics | Non-alcoholic Fatty Liver Disease - metabolism | Hypoglycemia - metabolism | Metabolism, Inborn Errors - genetics | Hyperglycemia - metabolism | Citric Acid Cycle - genetics | Animals | Gluconeogenesis - genetics | Glucose - metabolism | Mice | Metabolism, Inborn Errors - pathology | Enzymes | Carbohydrates | Liver diseases | Pathogenesis | Liver | Lipids | Glucose | Metabolism | Fatty acids | Studies | Nutrition research | Metabolites | Rodents | Diabetes | Index Medicus | Abridged Index Medicus
Journal Article
STEM CELLS, ISSN 1066-5099, 07/2013, Volume 31, Issue 7, pp. 1287 - 1297
In congenital mitochondrial DNA (mtDNA) disorders, a mixture of normal and mutated mtDNA (termed heteroplasmy) exists at varying levels in different tissues,... 
Pearson's marrow pancreas syndrome | Mitochondrial DNA | Induced pluripotent stem cells | Heteroplasmy | Hematopoiesis | Human genetics | MUSCLE | FIBROBLASTS | CELL & TISSUE ENGINEERING | CELL BIOLOGY | MTDNA | ONCOLOGY | GENETICS | BIOTECHNOLOGY & APPLIED MICROBIOLOGY | MUTATION | GENERATION | PATIENT | HEMATOLOGY | Mitochondrial Diseases - pathology | Sequence Deletion | Fibroblasts - physiology | Humans | Mitochondrial Diseases - metabolism | Child, Preschool | Anemia, Sideroblastic - metabolism | Cell Differentiation - genetics | DNA, Mitochondrial - genetics | Lipid Metabolism, Inborn Errors - pathology | Hematopoietic Stem Cells - physiology | Female | Anemia, Sideroblastic - pathology | Induced Pluripotent Stem Cells - cytology | Fibroblasts - metabolism | Induced Pluripotent Stem Cells - metabolism | Mitochondrial Diseases - genetics | Cell Line | DNA, Mitochondrial - metabolism | Induced Pluripotent Stem Cells - physiology | Lipid Metabolism, Inborn Errors - metabolism | Acyl-CoA Dehydrogenase, Long-Chain - metabolism | Muscular Diseases - metabolism | Lipid Metabolism, Inborn Errors - diagnosis | Hematopoietic Stem Cells - metabolism | Muscular Diseases - pathology | Anemia, Sideroblastic - genetics | Muscular Diseases - diagnosis | Hematopoietic Stem Cells - cytology | Fibroblasts - cytology | Mitochondrial Diseases - diagnosis | Acyl-CoA Dehydrogenase, Long-Chain - deficiency | Genetic research | Stem cell research | Genetic disorders | Analysis | Stem cells | Index Medicus
Journal Article
Journal of Physiology, ISSN 0022-3751, 2013, Volume 591, Issue 2, pp. 571 - 592
Creatine (Cr) plays an important role in muscle energy homeostasis by its participation in the ATP-phosphocreatine phosphoryl exchange reaction mediated by... 
GUANIDINOACETATE METHYLTRANSFERASE DEFICIENCY | RESPIRATORY-CHAIN | PHYSIOLOGY | MAGNETIC-RESONANCE-SPECTROSCOPY | ACTIVATED PROTEIN-KINASE | HUMAN BRAIN | IN-VIVO | ACETYL-COA CARBOXYLASE | NEUROSCIENCES | HUMAN SKELETAL-MUSCLE | BETA-GUANIDINOPROPIONIC ACID | ORAL SUPPLEMENTATION | Speech Disorders - diet therapy | Developmental Disabilities - metabolism | Amino Acid Metabolism, Inborn Errors - physiopathology | Amidinotransferases - genetics | Muscle, Skeletal - metabolism | Amino Acid Metabolism, Inborn Errors - diet therapy | Muscle Fibers, Skeletal - metabolism | Mitochondria - ultrastructure | Intellectual Disability - metabolism | Proton-Translocating ATPases - metabolism | Speech Disorders - pathology | Amino Acid Metabolism, Inborn Errors - metabolism | Developmental Disabilities - pathology | Adenosine Triphosphate - metabolism | Creatine - deficiency | Amino Acid Metabolism, Inborn Errors - pathology | Intellectual Disability - diet therapy | Hand Strength | Developmental Disabilities - physiopathology | Speech Disorders - metabolism | Amidinotransferases - deficiency | Magnetic Resonance Spectroscopy | Intellectual Disability - pathology | Ischemia - metabolism | Lipid Metabolism | Muscular Atrophy - genetics | Mitochondria - metabolism | Amidinotransferases - metabolism | Speech Disorders - physiopathology | Mice, Knockout | Intellectual Disability - physiopathology | Phosphates - metabolism | Animals | Energy Metabolism | Muscle, Skeletal - physiopathology | Hindlimb - pathology | Muscle Fibers, Skeletal - pathology | Creatine Kinase - metabolism | Creatine - therapeutic use | Mice | Muscle, Skeletal - pathology | Developmental Disabilities - diet therapy | Hydrogen-Ion Concentration | Physiological aspects | Creatine | Homeostasis | Enzymes | Musculoskeletal system | Biosynthesis | Metabolism | Morphology | Index Medicus | Skeletal Muscle and Exercise
Journal Article
Journal of Biological Chemistry, ISSN 0021-9258, 07/2014, Volume 289, Issue 28, pp. 19531 - 19537
Journal Article
PLoS ONE, ISSN 1932-6203, 2016, Volume 11, Issue 2, pp. e0147818 - e0147818
Rhabdomyolysis is common in very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) and other metabolic myopathies, but its pathogenic basis is poorly... 
SKELETAL-MUSCLE | MAGNETIC-RESONANCE-SPECTROSCOPY | ACID BETA-OXIDATION | MAXIMAL FAT OXIDATION | FATIGUE | MULTIDISCIPLINARY SCIENCES | IN-VIVO | KINASE | RESISTANCE | DUCHENNE MUSCULAR-DYSTROPHY | MITOCHONDRIAL MYOPATHIES | Mitochondrial Diseases - pathology | Humans | Muscle Fibers, Fast-Twitch - metabolism | Mitochondrial Diseases - metabolism | Rhabdomyolysis - complications | Male | Muscle Fibers, Slow-Twitch - metabolism | Adenosine Triphosphate - biosynthesis | Case-Control Studies | Mitochondrial Diseases - complications | Exercise | Lipid Metabolism, Inborn Errors - pathology | Muscular Diseases - physiopathology | Rhabdomyolysis - pathology | Adult | Female | Lipid Metabolism, Inborn Errors - metabolism | Acyl-CoA Dehydrogenase, Long-Chain - metabolism | Muscular Diseases - metabolism | Oxidative Phosphorylation | Rhabdomyolysis - physiopathology | Mitochondria - metabolism | Muscular Diseases - pathology | Models, Statistical | Acetylcarnitine - blood | Rhabdomyolysis - metabolism | Lipid Metabolism, Inborn Errors - physiopathology | Phosphates - metabolism | Muscle Fibers, Slow-Twitch - pathology | Adolescent | Muscle Fibers, Fast-Twitch - pathology | Muscular Diseases - complications | Phosphocreatine - metabolism | Lipid Metabolism, Inborn Errors - complications | Mitochondrial Diseases - physiopathology | Acyl-CoA Dehydrogenase, Long-Chain - deficiency | Physiological aspects | Cycling | Research | Rhabdomyolysis | Risk factors | Phosphates | Pediatrics | Neurosciences | Nuclear magnetic resonance--NMR | Laboratories | Oxidative metabolism | Homeostasis | Chains | Dehydrogenase | Muscular dystrophy | Acyl-CoA dehydrogenase | Defects | Mitochondria | Child development | Energy | Metabolites | Rodents | Workloads | Mathematical models | Bioinformatics | Enzymes | Departments | Phosphorus | Phosphocreatine | Earth science | Metabolism | Medical screening | Patients | Fatty acids | Carnitine | Children & youth | Energy balance | Musculoskeletal system | Chemistry | Hypotheses | Hospitals | Bicycles | Quadriceps muscle | Respiration | ATP | Metabolic disorders | Index Medicus | Nuclear magnetic resonance | NMR
Journal Article
Journal Article
Journal of Biological Chemistry, ISSN 0021-9258, 2017, Volume 292, Issue 45, pp. 18443 - 18456
Cardiac hypertrophy is closely linked to impaired fatty acid oxidation, but the molecular basis of this link is unclear. Here, we investigated the loss of an... 
MORTALITY | TARGET | PATHOLOGICAL HYPERTROPHY | cardiac metabolism | BIOCHEMISTRY & MOLECULAR BIOLOGY | mammalian target of rapamycin (mTOR) | HDAC INHIBITION | FAILURE | LEFT-VENTRICULAR HYPERTROPHY | DIASTOLIC DYSFUNCTION | acetylation | METABOLISM | FATTY-ACID OXIDATION | fatty acid oxidation | cardiac hypertrophy | CORONARY-HEART-DISEASE | Carnitine O-Palmitoyltransferase - genetics | Mitochondria, Heart - metabolism | Mitochondria, Heart - pathology | Atrial Remodeling - drug effects | Cardiomegaly - etiology | Male | Metabolism, Inborn Errors - therapy | Mitochondria, Heart - drug effects | Protein Processing, Post-Translational - drug effects | Carnitine O-Palmitoyltransferase - metabolism | Myocardium - metabolism | Carnitine O-Palmitoyltransferase - deficiency | Drug Resistance | Heart - physiopathology | Sirolimus - therapeutic use | Specific Pathogen-Free Organisms | Diet, Ketogenic | Metabolism, Inborn Errors - metabolism | Mice, Transgenic | Myocardium - pathology | Mechanistic Target of Rapamycin Complex 1 - antagonists & inhibitors | Enzyme Activation - drug effects | Mechanistic Target of Rapamycin Complex 1 - metabolism | Mice, Knockout | Acetylation - drug effects | Myocardium - enzymology | Animals | Cardiomegaly - prevention & control | Protein Kinase Inhibitors - therapeutic use | Survival Analysis | Heart - drug effects | Histone Deacetylase Inhibitors - therapeutic use | Metabolism, Inborn Errors - pathology | Metabolism, Inborn Errors - physiopathology | Crosses, Genetic | Index Medicus | Lipids
Journal Article