Page last updated: 2024-08-17

coenzyme a and Lipid Metabolism, Inborn Error

coenzyme a has been researched along with Lipid Metabolism, Inborn Error in 17 studies

Research

Studies (17)

TimeframeStudies, this research(%)All Research%
pre-199010 (58.82)18.7374
1990's1 (5.88)18.2507
2000's0 (0.00)29.6817
2010's1 (5.88)24.3611
2020's5 (29.41)2.80

Authors

AuthorsStudies
Cole, D; Darin, N; Donker Kaat, L; Doolaard, M; Ebberink, MS; Ferdinandusse, S; Fuchs, SA; Hedberg-Oldfors, C; Huidekoper, HH; Moat, SJ; Olpin, S; Régal, L; Ruiter, JPN; Schwantje, M; Visser, G1
Tucci, S1
Gokmen-Ozel, H; Kadayifcilar, S; Kahraman, AB; Sivri, S; Yildiz, Y1
Bakker, BM; Derks, TGJ; Gerding, A; Heiner-Fokkema, R; Huijkman, NCA; Jager, EA; Kiyuna, LA; Martines, AMF; Odendaal, C; van Eunen, K; Vieira-Lara, MA; Wolters, JC1
Krett, B; Straub, V; Vissing, J1
Alcorn, JF; Beck, ME; Bharathi, SS; Chen, R; Ellis, B; Goetzman, ES; Kagan, VE; Karunanidhi, A; Kosmider, B; Mallampalli, RK; McHugh, KJ; McKinney, RW; Otsubo, C; Skilling, H; Suhrie, KR; Tyurina, YY; Uppala, R; Vockley, J; Yeasted, R; Zuo, YY1
Mortensen, PB1
Corkey, BE; Hsu, BY; Lieu, YK; Price, WA; Stanley, CA1
Baymann, NA; Bourre, JM; Daudu, OL1
Baumann, NA; Bourre, JM; Daudu, OL; Paturneau-Jouas, MY1
Hähnel, R; Wysocki, SJ1
Baumann, NA; Bourre, JM; Daudu, OL1
Penn, D; Schmidt-Sommerfeld, E1
Gompertz, D1
Vaisrub, S1
Engel, PC1
Baumann, N; Bourre, JM; Daudu, O; Pollet, S1

Reviews

1 review(s) available for coenzyme a and Lipid Metabolism, Inborn Error

ArticleYear
Dicarboxylic acids and the lipid metabolism.
    Danish medical bulletin, 1984, Volume: 31, Issue:2

    Topics: Acyl Coenzyme A; Animals; Carboxylic Acids; Carnitine O-Acetyltransferase; Coenzyme A; Dicarboxylic Acids; Fatty Acids; Gas Chromatography-Mass Spectrometry; Hormones; Humans; Hydroxylation; Ketosis; Lipase; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Microbodies; Oxidation-Reduction; Rats; Valproic Acid

1984

Other Studies

16 other study(ies) available for coenzyme a and Lipid Metabolism, Inborn Error

ArticleYear
Thermo-sensitive mitochondrial trifunctional protein deficiency presenting with episodic myopathy.
    Journal of inherited metabolic disease, 2022, Volume: 45, Issue:4

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Adolescent; Cardiomyopathies; Child; Child, Preschool; Coenzyme A; Delayed Diagnosis; Fatty Acids; Humans; Lipid Metabolism, Inborn Errors; Mitochondrial Myopathies; Mitochondrial Trifunctional Protein; Muscular Diseases; Nervous System Diseases; Rhabdomyolysis

2022
An Altered Sphingolipid Profile as a Risk Factor for Progressive Neurodegeneration in Long-Chain 3-Hydroxyacyl-CoA Deficiency (LCHADD).
    International journal of molecular sciences, 2022, Jun-27, Volume: 23, Issue:13

    Topics: Coenzyme A; Fatty Acids; Humans; Lipid Metabolism, Inborn Errors; Mitochondrial Trifunctional Protein; Risk Factors; Sphingolipids

2022
Successful management of rhabdomyolysis with triheptanoin in a child with severe long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency.
    Neuromuscular disorders : NMD, 2023, Volume: 33, Issue:4

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Child; Coenzyme A; Female; Humans; Infant; Lipid Metabolism, Inborn Errors; Oxidation-Reduction; Rhabdomyolysis; Triglycerides

2023
Personalised modelling of clinical heterogeneity between medium-chain acyl-CoA dehydrogenase patients.
    BMC biology, 2023, 09-04, Volume: 21, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Coenzyme A; Humans; Lipid Metabolism; Lipid Metabolism, Inborn Errors

2023
Episodic hyperCKaemia may be a feature of α-methylacyl-coenzyme A racemase deficiency.
    European journal of neurology, 2021, Volume: 28, Issue:2

    Topics: Adult; Coenzyme A; Humans; Lipid Metabolism, Inborn Errors; Racemases and Epimerases

2021
Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction.
    The Journal of biological chemistry, 2014, Apr-11, Volume: 289, Issue:15

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adult; Animals; Bronchi; Cell Line, Tumor; Coenzyme A; Disease Models, Animal; Epithelial Cells; Fatty Acids; Female; Homozygote; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Lung; Lung Diseases; Lung Neoplasms; Male; Mice; Mice, Inbred C57BL; Mice, Transgenic; Oxygen; Phosphatidylcholines; Phosphatidylglycerols; Polymorphism, Genetic; Pulmonary Alveoli; Pulmonary Surfactants

2014
Carnitine effects on coenzyme A profiles in rat liver with hypoglycin inhibition of multiple dehydrogenases.
    The American journal of physiology, 1997, Volume: 272, Issue:3 Pt 1

    Topics: Acyl-CoA Dehydrogenases; Animals; Carnitine; Coenzyme A; Cyclopropanes; Enzyme Inhibitors; Hypoglycins; Ketones; Lipid Metabolism, Inborn Errors; Liver; Mitochondria, Liver; Rats; Rats, Sprague-Dawley; Spiro Compounds

1997
Biosynthesis of lignoceric acid from behenyl0COA in mouse brain microsomes. Comparison between normal and Quaking mutant.
    Biochemical and biophysical research communications, 1975, Apr-21, Volume: 63, Issue:4

    Topics: Animals; Brain; Chromatography, Gas; Chromatography, Thin Layer; Coenzyme A; Fatty Acids; Kinetics; Lipid Metabolism, Inborn Errors; Malonates; Mice; Microsomes; Mutation; Myelin Sheath; NADP; Oxidation-Reduction; Phospholipids; Time Factors

1975
Lignoceric acid biosynthesis in the developing brain. Activities of mitochondrial acetyl-CoA-dependent synthesis and microsomal malonyl-CoA chain-elongating system in relation to myelination. Comparison between normal mouse and dysmyelinating mutants (qua
    European journal of biochemistry, 1977, Jan-03, Volume: 72, Issue:1

    Topics: Aging; Animals; Brain; Coenzyme A; Fatty Acid Synthases; Fatty Acids; Kinetics; Lipid Metabolism, Inborn Errors; Male; Malonates; Mice; Mice, Inbred C57BL; Microsomes; Mitochondria; Mutation; Myelin Sheath; Species Specificity

1977
3-Hydroxy-3-methylglutaric aciduria: deficiency of 3-hydroxy-3-methylglutaryl coenzyme A lyase.
    Clinica chimica acta; international journal of clinical chemistry, 1976, Sep-06, Volume: 71, Issue:2

    Topics: Coenzyme A; Fibroblasts; Glutarates; Hexosaminidases; Humans; Hydroxy Acids; Lipid Metabolism, Inborn Errors; Oxo-Acid-Lyases

1976
Fatty acid biosynthesis in mice brain and kidney microsomes: comparison between quaking mutant and control.
    Journal of neurochemistry, 1975, Volume: 24, Issue:5

    Topics: Animals; Brain; Coenzyme A; Fatty Acids; Kidney; Lipid Metabolism, Inborn Errors; Malonates; Mice; Microsomes; Mutation; Myelin Sheath; Palmitic Acids; Phospholipids; Stearic Acids

1975
[Carnitine deficiency].
    Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde, 1986, Volume: 134, Issue:5

    Topics: Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Child; Coenzyme A; Cytochrome-c Oxidase Deficiency; Fatty Acid Desaturases; Fatty Acids; Humans; Ketones; Lipid Metabolism, Inborn Errors; Mitochondria, Liver; Mitochondria, Muscle; Muscular Diseases; Renal Aminoacidurias; Renal Dialysis; Transferases

1986
The distribution of 17 carbon fatty acids in the liver of a child with propionicacidaemia.
    Lipids, 1971, Volume: 6, Issue:8

    Topics: Animals; Chemical Phenomena; Chemistry; Child; Cholesterol; Chromatography, Gas; Chromatography, Thin Layer; Coenzyme A; Esters; Fatty Acids; Fatty Acids, Essential; Fatty Acids, Nonesterified; Humans; Ligases; Lipase; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Liver; Male; Oxidation-Reduction; Pancreas; Periodic Acid; Phosphatidylcholines; Phosphatidylethanolamines; Phospholipases; Propionates; Snakes; Swine; Triglycerides; Venoms

1971
Carnitine deficiency myopathy.
    JAMA, 1973, Jul-09, Volume: 225, Issue:2

    Topics: Adolescent; Carnitine; Coenzyme A; Female; Humans; Lipid Metabolism, Inborn Errors; Mitochondria, Muscle; Muscles; Muscular Diseases

1973
Possibility of inborn defect in isovalericacidaemia involving altered enzyme specificity rather than total inactivity.
    Nature, 1974, Mar-08, Volume: 248, Issue:5444

    Topics: Alanine; Butyrates; Coenzyme A; Cyclopropanes; Humans; Kinetics; Leucine; Lipid Metabolism, Inborn Errors; Oxidoreductases; Plants; Toxins, Biological; Valerates

1974
[Biosynthesis of fatty acids in brain microsomes of normal and quaking mice].
    Comptes rendus hebdomadaires des seances de l'Academie des sciences. Serie D: Sciences naturelles, 1971, Oct-27, Volume: 273, Issue:17

    Topics: Animals; Brain; Carbon Isotopes; Coenzyme A; Demyelinating Diseases; Fatty Acids; In Vitro Techniques; Lipid Metabolism, Inborn Errors; Malonates; Mice; Microsomes; Mutation; Palmitic Acids; Rodent Diseases; Stearic Acids; Tremor

1971