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JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, ISSN 0334-018X, 10/2019, Volume 32, Issue 10, pp. 1181 - 1185
Mitochondrial acyl-CoA dehydrogenase 9 (ACAD9) deficiency is one of the common causes of respiratory chain complex I deficiency, which is characterized by... 
treatment | THERAPY | deficiency | mitochondria | ACAD9 | ENDOCRINOLOGY & METABOLISM | PEDIATRICS | MUTATIONS | cardiomyopathy | pyruvate
Journal Article
Seminars in Cell and Developmental Biology, ISSN 1084-9521, 04/2018, Volume 76, pp. 154 - 162
Mitochondrial complex I is the primary entry point for electrons into the electron transport chain, required for the bulk of cellular ATP production via... 
Mitochondria | Membrane protein | Complex I | Oxidative phosphorylation | Assembly factor | BETA-OXIDATION | OXIDATIVE-PHOSPHORYLATION | PROTEIN | ENZYME-ACTIVITY | MEMBRANE | DEVELOPMENTAL BIOLOGY | DEFICIENCY | FOXRED1 | CELL BIOLOGY | MOLECULAR CHAPERONE | ACAD9 MUTATIONS | FAD-DEPENDENT OXIDOREDUCTASE
Journal Article
JIMD Reports, ISSN 2192-8304, 2016, Volume 28, pp. 1 - 10
ACAD9 (acyl-CoA dehydrogenase 9) is an essential factor for the mitochondrial respiratory chain complex I assembly. ACAD9, a member of acyl-CoA dehydrogenase... 
β-oxidation | acad9 | Fetal cardiomegaly | Mitochondrial respiratory chain | Chemical Sciences
Journal Article
The American Journal of Human Genetics, ISSN 0002-9297, 06/2012, Volume 90, Issue 6, pp. 1079 - 1087
Journal Article
Mitochondrion, ISSN 1567-7249, 05/2019, Volume 46, pp. 91 - 96
Assembly of complex I of the mitochondrial respiratory chain (MRC) requires not only structural subunits for electron transport, but also assembly factors. In... 
ACDH-12 | C. elegans | Very long-chain acyl-CoA dehydrogenase | Complex I assembly factor | ACAD9 | RESPIRATORY-CHAIN | OXIDATIVE-PHOSPHORYLATION | LIFE-SPAN | DAMAGE | CELL BIOLOGY | ACYL-COA DEHYDROGENASE | GENETICS & HEREDITY | MUTATIONS | EXPRESSION | Caenorhabditis elegans | Amino acids | Nematoda | Fatty acids | Analysis
Journal Article
The American Journal of Human Genetics, ISSN 0002-9297, 03/2018, Volume 102, Issue 3, pp. 460 - 467
Journal Article
PLoS ONE, ISSN 1932-6203, 08/2016, Volume 11, Issue 8, p. e0156738
Background & Aims The etiology of acute liver failure (ALF) remains elusive in almost half of affected children. We hypothesized that inherited mitochondrial... 
DIAGNOSIS | MITOCHONDRIAL-DNA DEPLETION | RIBONUCLEOTIDE REDUCTASE SUBUNIT | MULTIDISCIPLINARY SCIENCES | DISEASE | ACAD9 | CHOLESTASIS | COMPLEX I DEFICIENCY | MOLECULAR CHARACTERISTICS | MUTATIONS | P53R2 | Liver - pathology | Humans | Liver - metabolism | Child, Preschool | Infant | Male | Liver Failure, Acute - metabolism | Mitochondria - metabolism | Mitochondria - pathology | Genetic Variation | DNA, Mitochondrial - genetics | Energy Metabolism | Mitochondria - genetics | Liver Failure, Acute - genetics | Liver Failure, Acute - pathology | Adolescent | Female | High-Throughput Nucleotide Sequencing | Retrospective Studies | Mutation | Child | Infant, Newborn | Energy metabolism | Liver failure | Care and treatment | Bioenergetics | Patient outcomes | Mitochondrial diseases | Research | Risk factors | Pediatrics | Phosphorylation | Dehydrogenases | Disease | Liver | Genes | Insertion | Pyruvic acid | Mitochondrial DNA | Gene sequencing | Mitochondria | Ribonucleotide reductase | Metabolites | Etiology | Hepatology | Gastroenterology | Genetics | Oxidation | Children | Lactic acidosis | Deoxyribonucleic acid--DNA | Liver diseases | Biochemical analysis | Nutrition | Metabolism | Electron microscopy | Patients | Fatty acids | Steatosis | Genetic variance | Pathology | Hospitals | Hepatocytes | Nitric oxide | Lactic acid | Electron transport | Acidosis | Deoxyribonucleic acid | DNA
Journal Article
The British journal of nutrition, ISSN 0007-1145, 2017, Volume 118, Issue 9, pp. 641 - 650
Suboptimal vitamin B2 status is encountered globally. Riboflavin deficiency depresses growth and results in a fatty liver. The underlying mechanisms remain to... 
TCA tricarboxylic acid | FXN frataxin | HMGCS1 hydroxymethylglutaryl-CoA synthase | RD riboflavin-deficient diet | Electron transport chain | pair-fed with the control diet to the mean daily intake of the riboflavin-deficient group | ACAT2 acetyl-CoA acetyltransferase | NDUFS1 NADH-ubiquinone oxidoreductase 75 kDa subunit | Lipid metabolism | β-Oxidation | ACADM medium-chain specific acyl-CoA dehydrogenase | SCP2 non-specific lipid-transfer protein | TC total cholesterol | DLD dihydrolipoyl dehydrogenase | MDH1 malate dehydrogenase | MTTP microsomal TAG transfer protein | ETC electron transport chain | Riboflavin deficiency | Liver proteomics | NDUFA8 NADH dehydrogenase (ubiquinone) 1α subcomplex subunit 8 | CPF | ACAD9 acyl-CoA dehydrogenase family member 9 | ACADS short-chain specific acyl-CoA dehydrogenase | APOB apo B-100 CAL fed ad libitum a control diet | ETFDH electron transfer flavoprotein-ubiquinone oxidoreductase | LIPID-METABOLISM | beta-Oxidation | FOOD UTILIZATION | MITOCHONDRIAL OXIDATIVE-METABOLISM | BROWN ADIPOSE-TISSUE | RAT-LIVER MITOCHONDRIA | GENE ONTOLOGY | PYRUVATE-DEHYDROGENASE COMPLEX | GROWTH-PERFORMANCE | NUTRITION & DIETETICS | COMPLEX-I-DEFICIENCY | ANTIOXIDANT STATUS | Neurosciences | Phosphorylation | Dehydrogenases | Growth rate | Liver | Riboflavin | Lipids | Biosynthesis | Mental depression | Aquatic birds | Accumulation | Western blotting | Proteins | Mitochondria | Alterations | Fatty liver | Vitamin B | Oxidation | Age | Enlargement | Enzymes | Liver diseases | Nutrient deficiency | Waterfowl | Birds | Metabolism | Fatty acids | Cholesterol | Diet | Mutation | Feeds | Electron transport | Metabolic disorders
Journal Article