Page last updated: 2024-08-17

coenzyme a and Inborn Errors of Metabolism

coenzyme a has been researched along with Inborn Errors of Metabolism in 27 studies

Research

Studies (27)

Timeframe	Studies, this research(%)	All Research%
pre-1990	22 (81.48)	18.7374
1990's	2 (7.41)	18.2507
2000's	0 (0.00)	29.6817
2010's	3 (11.11)	24.3611
2020's	0 (0.00)	2.80

Authors

Authors	Studies
Carecchio, M; Di Meo, I; Tiranti, V	1
Dusi, S; Tiranti, V; Valletta, L; Venco, P	1
Bian, F; Brunengraber, H; Jin, Z; Kelleher, JK; Tomcik, K; Zhang, GF	1
Stewart, PM; Walser, M	1
Brass, EP	1
Fromenty, B; Pessayre, D	1
Britt, W; Giles, H; Lebowitz, J; Morrow, G; Revsin, B	1
Wolf, B	1
Brandt, NJ; Brandt, S; Christensen, E; Gregersen, N; Rasmussen, K	1
Brandt, NJ; Christensen, E	1
Draye, JP; Vamecq, J	1
Arts, WF; Busch, HF; Luyt-Houwen, IE; Przyrembel, H; Scholte, HR; Vaandrager-Verduin, MH	1
Engel, AG; Frens, D; Mack, D; Ogasahara, S	1
Ferguson-Smith, MA; Ferguson-Smith, ME; Gompertz, D; Goodey, PA; MacLean, MV; Russell, G; Thom, H	1
Callaghan, P; Newman, CG; Wilson, BD; Young, L	1
Cornblath, M; Tildon, JT	1
Charpentier, C; Chignolle, A; Girard, S; Gompertz, D; Goodey, PA; Saudubray, JM	1
Tildon, JT	1
Gompertz, D	1
Gerald, PS; Kang, ES; Snodgrass, PJ	1
Gompertz, D; Goodey, PA	1
Ando, T; Bachmann, C; Nyhan, WL; Rasmussen, K; Scott, R; Smith, EK	1
Ando, T; Nyhan, WL; Rasmussen, K; Wright, JM	1
Ando, T; Cottom, D; Hull, D; Kilroy, AW; Nyhan, WL; Rasmussen, K; Wadlington, W	2
Gompertz, D; Mitropoulos, KA; Myant, NB	1
Hsia, YE; Lilljeqvist, A; Rosenberg, LE	1

Reviews

5 review(s) available for coenzyme a and Inborn Errors of Metabolism

Article	Year
Inborn errors of coenzyme A metabolism and neurodegeneration. Journal of inherited metabolic disease, 2019, Volume: 42, Issue:1 Topics: Animals; Brain; Coenzyme A; Humans; Iron; Metabolism, Inborn Errors; Mitochondria; Mutation; Nervous System; Neurodegenerative Diseases; Phosphotransferases (Alcohol Group Acceptor)	2019
Alteration of the coenzyme A biosynthetic pathway in neurodegeneration with brain iron accumulation syndromes. Biochemical Society transactions, 2014, Volume: 42, Issue:4 Topics: Animals; Brain; Coenzyme A; Humans; Iron; Metabolism, Inborn Errors; Mitochondria; Neurodegenerative Diseases; Phosphotransferases (Alcohol Group Acceptor)	2014
Overview of coenzyme A metabolism and its role in cellular toxicity. Chemico-biological interactions, 1994, Volume: 90, Issue:3 Topics: Acyl Coenzyme A; Animals; Biotransformation; Coenzyme A; Humans; Liver; Metabolism, Inborn Errors; Methylmalonic Acid; Xenobiotics	1994
Impaired mitochondrial function in microvesicular steatosis. Effects of drugs, ethanol, hormones and cytokines. Journal of hepatology, 1997, Volume: 26 Suppl 2 Topics: Animals; Coenzyme A; Cytokines; DNA, Mitochondrial; Electron Transport; Ethanol; Fatty Liver; Hormones; Humans; Metabolism, Inborn Errors; Mitochondria, Liver; Oxidation-Reduction	1997
Inborn errors of organic acid metabolism. Clinics in endocrinology and metabolism, 1974, Volume: 3, Issue:1 Topics: Adult; Amino Acid Metabolism, Inborn Errors; Biotin; Carboxylic Acids; Chromatography, Gas; Coenzyme A; Crotonates; Female; Gestational Age; Humans; Hydroxy Acids; Hydroxybutyrates; Infant; Infant, Newborn; Isomerases; Leucine; Male; Malonates; Metabolism, Inborn Errors; Pedigree; Pregnancy; Prenatal Diagnosis; Propionates; Pyrrolidonecarboxylic Acid; Valerates; Vitamin B 12	1974

Other Studies

22 other study(ies) available for coenzyme a and Inborn Errors of Metabolism

Article	Year
Compartmentation of Metabolism of the C12-, C9-, and C5-n-dicarboxylates in Rat Liver, Investigated by Mass Isotopomer Analysis: ANAPLEROSIS FROM DODECANEDIOATE. The Journal of biological chemistry, 2015, Jul-24, Volume: 290, Issue:30 Topics: Amino Acid Metabolism, Inborn Errors; Animals; Citric Acid Cycle; Coenzyme A; Dicarboxylic Acids; Fatty Acids; Glutarates; Humans; Liver; Malonyl Coenzyme A; Metabolism, Inborn Errors; Mitochondria; Myocardium; Oxidation-Reduction; Peroxisomes; Propionic Acidemia; Rats	2015
Organic acidaemia and Hyperammonaemia: review. Journal of inherited metabolic disease, 1981, Volume: 4, Issue:4 Topics: Acetyl Coenzyme A; Ammonia; Animals; Carbamoyl-Phosphate Synthase (Ammonia); Carboxylic Acids; Coenzyme A; Glutamates; Humans; Metabolism, Inborn Errors; Mitochondria, Liver; Rats	1981
Detection of errors in methylmalonyl-CoA metabolism by using amniotic fluid. Clinical chemistry, 1977, Volume: 23, Issue:5 Topics: Amniotic Fluid; Cells, Cultured; Coenzyme A; False Positive Reactions; Female; Humans; Malonates; Metabolism, Inborn Errors; Methods; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Pregnancy; Pregnancy Trimester, Second; Prenatal Diagnosis	1977
Evaluation of biotin responsiveness in cultured fibroblasts from patients with propionic acidemia: absence of response by structurally altered carboxylases. Biochemical genetics, 1979, Volume: 17, Issue:7-8 Topics: Biotin; Carboxy-Lyases; Coenzyme A; Fibroblasts; Humans; Metabolism, Inborn Errors; Propionates; Stimulation, Chemical	1979
Glutaric aciduria in progressive choreo-athetosis. Clinical genetics, 1978, Volume: 13, Issue:1 Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Athetosis; Cerebral Palsy; Child; Coenzyme A; Female; Glutarates; Humans; Metabolism, Inborn Errors; Oxidoreductases	1978
Studies on glutaryl-CoA dehydrogenase in leucocytes, fibroblasts and amniotic fluid cells. The normal enzyme and the mutant form in patients with glutaric aciduria. Clinica chimica acta; international journal of clinical chemistry, 1978, Sep-01, Volume: 88, Issue:2 Topics: Amniotic Fluid; Cells, Cultured; Coenzyme A; Female; Fibroblasts; Glutarates; Humans; Kinetics; Leukocytes; Male; Metabolism, Inborn Errors; Mutation; Oxidoreductases; Pedigree; Pregnancy	1978
Comparison between the formation and the oxidation of dicarboxylylcarnitine esters in rat liver and skeletal muscle: possible implications for human inborn disorders of mitochondrial beta-oxidation. Journal of inherited metabolic disease, 1989, Volume: 12, Issue:1 Topics: Animals; Coenzyme A; Dicarboxylic Acids; Humans; Male; Metabolism, Inborn Errors; Mitochondria, Liver; Mitochondria, Muscle; Oxidation-Reduction; Rats; Rats, Inbred Strains	1989
Defects in oxidative phosphorylation. Biochemical investigations in skeletal muscle and expression of the lesion in other cells. Journal of inherited metabolic disease, 1987, Volume: 10 Suppl 1 Topics: Adolescent; Adult; Biotin; Brain; Carnitine; Cells, Cultured; Child, Preschool; Coenzyme A; Cytochrome-c Oxidase Deficiency; Electron Transport Complex III; Female; Fibroblasts; Humans; Infant; Male; Metabolism, Inborn Errors; Mitochondria; Mitochondria, Liver; Mitochondria, Muscle; Mitochondrial ADP, ATP Translocases; NAD(P)H Dehydrogenase (Quinone); Oxidative Phosphorylation; Quinone Reductases	1987
Muscle coenzyme Q deficiency in familial mitochondrial encephalomyopathy. Proceedings of the National Academy of Sciences of the United States of America, 1989, Volume: 86, Issue:7 Topics: Adolescent; Child; Coenzyme A; Cytochromes; Electron Transport; Female; Humans; Metabolism, Inborn Errors; Mitochondria, Muscle; Neuromuscular Diseases; Oxygen Consumption; Reference Values; Ubiquinone	1989
Antenatal diagnosis of propianicacidaemia. Lancet (London, England), 1973, May-05, Volume: 1, Issue:7810 Topics: Amino Acids; Amniocentesis; Amniotic Fluid; Biotin; Carboxy-Lyases; Coenzyme A; Diet Therapy; Female; Humans; Infant, Newborn; Metabolism, Inborn Errors; Pregnancy; Prenatal Diagnosis; Propionates	1973
Neonatal death associated with isovalericacidaemia. Lancet (London, England), 1967, Aug-26, Volume: 2, Issue:7513 Topics: Acidosis; Bicarbonates; Chromatography; Chromatography, Gas; Coenzyme A; Electrolytes; Fatty Acids; Fatty Acids, Nonesterified; Glucose; Hemorrhage; Humans; Hypocalcemia; Infant Mortality; Infant, Newborn; Leucine; Male; Metabolism, Inborn Errors; Valerates	1967
Succinyl-CoA: 3-ketoacid CoA-transferase deficiency. A cause for ketoacidosis in infancy. The Journal of clinical investigation, 1972, Volume: 51, Issue:3 Topics: Acidosis; Acyltransferases; Adipates; Autopsy; Brain; Cell Fractionation; Coenzyme A; Culture Techniques; Fibroblasts; Glucose; Glyceraldehyde-3-Phosphate Dehydrogenases; Glycolysis; Humans; Infant, Newborn; Keto Acids; Kidney; Male; Metabolism, Inborn Errors; Muscles; Phosphofructokinase-1; Phosphopyruvate Hydratase; Pyruvate Kinase; Skin; Succinates; Sulfurtransferases	1972
Prenatal diagnosis of methylmalonic aciduria. Pediatrics, 1974, Volume: 54, Issue:4 Topics: Adult; Amniotic Fluid; Chromatography, Gas; Coenzyme A; Female; Humans; Infant, Newborn; Isomerases; Male; Malonates; Metabolism, Inborn Errors; Pregnancy; Prenatal Diagnosis	1974
An alteration in glucose metabolism associated with a defect in ketone body metabolism. Proceedings of the National Academy of Sciences of the United States of America, 1973, Volume: 70, Issue:1 Topics: Carbohydrate Metabolism, Inborn Errors; Carbon Dioxide; Carbon Isotopes; Cells, Cultured; Citric Acid Cycle; Coenzyme A; Culture Media; Fibroblasts; Glucose; Glycolysis; Humans; Ketone Bodies; Metabolism, Inborn Errors; Propionates; Puromycin; Pyruvates; Succinates; Sulfurtransferases	1973
Methylmalonyl coenzyme A racemase defect: another cause of methylmalonic aciduria. Pediatric research, 1972, Volume: 6, Issue:12 Topics: Acidosis; Ammonia; Carbon Isotopes; Cells, Cultured; Cobamides; Coenzyme A; Fibroblasts; Humans; Infant, Newborn; Isomerases; Liver; Male; Malonates; Metabolism, Inborn Errors; Mitochondria, Liver; Propionates; Succinates; Tritium	1972
Methylmalonyl CoA mutase--a radiochromatographic assay. Clinica chimica acta; international journal of clinical chemistry, 1972, Volume: 42, Issue:1 Topics: Adult; Carbon Isotopes; Child; Chromatography, Gas; Cobamides; Coenzyme A; Enzyme Activation; Humans; Isomerases; Malonates; Metabolism, Inborn Errors; Methods; Succinates	1972
Isovaleric acidemia: identification of isovalerate, isovalerylglycine, and 3-hydroxyisovalerate in urine of a patient previously reported as having butyric and hexanoic acidemia. The Journal of pediatrics, 1973, Volume: 82, Issue:2 Topics: Butyrates; Caproates; Child; Chromatography, Gas; Chromatography, Thin Layer; Coenzyme A; Glycine; Humans; Mass Spectrometry; Metabolism, Inborn Errors; Valerates	1973
Isolation and identification of methylcitrate, a major metabolic product of propionate in patients with propionic acidemia. The Journal of biological chemistry, 1972, Apr-10, Volume: 247, Issue:7 Topics: Acids; Carbon Isotopes; Chromatography; Chromatography, Gas; Chromatography, Paper; Citrates; Coenzyme A; Humans; Malonates; Mass Spectrometry; Metabolism, Inborn Errors; Oxaloacetates; Propionates	1972
Tiglicaciduria in propionicacidaemia. The Biochemical journal, 1972, Volume: 126, Issue:4 Topics: Acrylates; Butyrates; Carbon Isotopes; Chromatography, Gas; Coenzyme A; Humans; Hydro-Lyases; Mass Spectrometry; Metabolism, Inborn Errors; Oxidation-Reduction; Propionates	1972
Excretion of tiglylglycine in propionic acidemia. The Journal of pediatrics, 1972, Volume: 81, Issue:5 Topics: Butyrates; Cells, Cultured; Chromatography, Gas; Coenzyme A; Diet Therapy; Fibroblasts; Glycine; Humans; Ligases; Mass Spectrometry; Metabolism, Inborn Errors; Methylation; Propionates	1972
The formation of propionate from the side-chain of cholesterol in an infant with an inborn error in the metabolism of propionate. The Biochemical journal, 1970, Volume: 118, Issue:3 Topics: Cholesterol; Coenzyme A; Humans; Infant, Newborn; Ligases; Liver; Male; Metabolism, Inborn Errors; Propionates	1970
Methylmalonic aciduria: metabolic block localization and vitamin B 12 dependency. Science (New York, N.Y.), 1968, Nov-15, Volume: 162, Issue:3855 Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Carbon Dioxide; Carbon Isotopes; Coenzyme A; Humans; Hydro-Lyases; Infant; Isomerases; Leukocytes; Malonates; Metabolism, Inborn Errors; Molecular Biology; Mutation; Propionates; Pyridoxal Phosphate; Succinates; Vitamin B 12; Xanthurenates	1968