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Nature Genetics, ISSN 1061-4036, 09/2012, Volume 44, Issue 9, pp. 1040 - 1045
Journal Article
The American Journal of Human Genetics, ISSN 0002-9297, 09/2013, Volume 93, Issue 3, pp. 482 - 495
Journal Article
PLoS ONE, ISSN 1932-6203, 07/2013, Volume 8, Issue 7, pp. e69282 - e69282
Primary mitochondrial respiratory chain (RC) diseases are heterogeneous in etiology and manifestations but collectively impair cellular energy metabolism.... 
SKELETAL-MUSCLE | OXIDATIVE STRESS | MUTANT MICE | IN-VITRO | ENERGY HOMEOSTASIS | HUMAN-CELLS | MULTIDISCIPLINARY SCIENCES | MITOCHONDRIAL-FUNCTION | ZINC-FINGER PROTEIN | GENE-EXPRESSION | BINDING SITES | Mitochondrial Diseases - genetics | Cell Line | Electron Transport - genetics | Humans | Middle Aged | 3' Untranslated Regions - genetics | Child, Preschool | Infant | Male | Muscle, Skeletal - metabolism | Gene Expression Profiling | Signal Transduction - genetics | Transcriptome - genetics | Young Adult | Adolescent | Adult | Female | Signal Transduction - physiology | Aged | Electron Transport - physiology | In Vitro Techniques | Mitochondrial Diseases - physiopathology | Child | Fibroblasts - metabolism | Physiological aspects | Development and progression | Mitochondrial diseases | Genetic aspects | Cellular signal transduction | Research | Genetic transcription | Sirtuins | Post-transcription | Pediatrics | Energy metabolism | Pathogenesis | Physicians | Nicotinic acid | Chains | Proteins | Mitochondria | NADH | RNA transport | Child development | Cellular communication | Etiology | Fibroblasts | Genetics | Forkhead protein | Nutrients | Protein transport | RNA processing | Complications | Muscles | Gene expression | Metabolism | Ribonucleic acid--RNA | Skeletal muscle | Children & youth | Medicine | NAD | Musculoskeletal system | Signaling | Hospitals | Ribosomal proteins | Human performance | Nicotinamide adenine dinucleotide | Nutrient availability | Peroxisome proliferator-activated receptors | Mutation | Electron transport | Detection | Metabolic disorders | Index Medicus | RNA | Ribonucleic acid
Journal Article
Journal of Inherited Metabolic Disease, ISSN 0141-8955, 3/2018, Volume 41, Issue 2, pp. 157 - 168
Propionic acidemia (PA) is a classical inborn error of metabolism with high morbidity that results from the inability of the propionyl-CoA carboxylase (PCC)... 
Human Genetics | Biochemistry, general | Pediatrics | C. elegans | Mitochondria | Medicine & Public Health | Propionic aciduria | Oxidative phosphorylation | TCA cycle | Metabolic Diseases | Internal Medicine | Propionic acidemia | MEDICINE, RESEARCH & EXPERIMENTAL | MANAGEMENT | INTERMEDIARY METABOLISM | SENSITIVITY | PYRUVATE-DEHYDROGENASE COMPLEX | SKELETAL-MUSCLE | ACIDEMIA PATIENTS | MUTATION | ACIDURIA | ENDOCRINOLOGY & METABOLISM | GENETICS & HEREDITY | DYSFUNCTION | Mitochondria - enzymology | Propionic Acidemia - enzymology | Genetic Predisposition to Disease | Caenorhabditis elegans - metabolism | Caenorhabditis elegans - genetics | Oxidative Stress - genetics | Caenorhabditis elegans Proteins - metabolism | Propionic Acidemia - genetics | Longevity - genetics | Methylmalonyl-CoA Decarboxylase - metabolism | Phenotype | Animals | Mitochondria - genetics | Gene Deletion | Membrane Potential, Mitochondrial - genetics | Energy Metabolism - genetics | Methylmalonyl-CoA Decarboxylase - genetics | Caenorhabditis elegans Proteins - genetics | Caenorhabditis elegans - enzymology | Disease Models, Animal | Physiological aspects | Caenorhabditis elegans | Wildlife conservation | Analysis | Tricarboxylic acid cycle | Oxidative stress | Energy metabolism | Phosphorylation | Inborn errors of metabolism | Oxidative metabolism | Amino acids | Quantitation | Gene deletion | Propionyl-CoA carboxylase | Metabolic flux | Clonal deletion | Polarography | Membrane potential | Glutathione | Enzymes | Metabolism | Morbidity | Citric acid | Life span | Oxalic acid | Nematodes | Lactic acid | Methylmalonyl-CoA | Index Medicus | propionic aciduria | propionic acidemia | mitochondria | oxidative phosphorylation
Journal Article
Molecular genetics and metabolism, ISSN 1096-7192, 4/2018, Volume 123, Issue 4, pp. 449 - 462
Oxidative stress is a known contributing factor in mitochondrial respiratory chain (RC) disease pathogenesis. Yet, no efficient means exists to objectively... 
C. elegans | fibroblasts | antioxidant | mitochondria | therapeutic modeling | zebrafish | genetic disease
Journal Article
Journal Article
EMBO Molecular Medicine, ISSN 1757-4676, 07/2011, Volume 3, Issue 7, pp. 410 - 427
Journal Article
HUMAN MOLECULAR GENETICS, ISSN 0964-6906, 06/2019, Volume 28, Issue 11, pp. 1837 - 1852
Cysteamine bitartrate is a US Food and Drug Administration-approved therapy for nephropathic cystinosis also postulated to enhance glutathione biosynthesis. We... 
PROTEIN | DNA | BIOCHEMISTRY & MOLECULAR BIOLOGY | GENETICS & HEREDITY | SENSITIVITY | MECHANISMS | DYSFUNCTION
Journal Article