carnitine and octanoylcarnitine

carnitine has been researched along with octanoylcarnitine in 79 studies

Research

Studies (79)

TimeframeStudies, this research(%)All Research%
pre-199021 (26.58)18.7374
1990's13 (16.46)18.2507
2000's13 (16.46)29.6817
2010's29 (36.71)24.3611
2020's3 (3.80)2.80

Authors

AuthorsStudies
Amatruda, JM; Kiesow, LA; Lockwood, DH; Margolis, S1
Harper, RD; Saggerson, ED1
Cockburn, F; Farquharson, J; Jamieson, EC; Logan, RW; Muir, J1
Gage, DA; Huang, ZH; Kossak, D; Li, BU; Penn, D; Rinaldo, P; Schmidt-Sommerfeld, E1
Gnoni, GV; Muci, MR1
Arrigoni Martelli, E; Bassani, E; Cardace, G; Corbelletta, C; Mancinelli, A; Marzo, A; Solbiati, M1
Bhuiyan, AK; Pande, SV1
Squire, RS1
Millington, DS; Roe, CR1
Brass, EP1
Bieber, LL; Kerner, J; Lebenthal, E; Penn, D; Rossi, TM; Schmidt-Sommerfeld, E1
Maltby, DA; Millington, DS; Roe, CR; Wellman, RB1
Bohan, TP; Liberato, DJ; Millington, DS; Roe, CR; Yergey, AL1
Kinnebrew, P; Maltby, DA; Millington, DS; Roe, CR1
Bohan, TP; Chalmers, RA; Kahler, SG; Maltby, DA; Millington, DS; Roe, CR1
Bruinvis, L; de Jager, JP; de Klerk, JB; Duran, M; Hofkamp, M; Ketting, D; Mitchell, G; Saudubray, JM; Wadman, SK1
Beckeringh, TE; Bruinvis, L; Dorland, L; Duran, M; Ketting, D; van Vossen, R; Wadman, SK1
Foster, DW; McGarry, JD1
Chalmers, RA; Hoppel, CL; Roe, CR; Stacey, TE1
Dorgan, DR; Kim, CS; Roe, CR1
Kawashima, S; Nakatani, T; Ozawa, K; Tobe, T; Yasuda, K1
Asano, M; Kamiyama, Y; Nakatani, T; Ozawa, K; Tobe, T; Ukikusa, M2
Arinze, IJ; Parameswaran, M1
Davis, EJ; Davis-van Thienen, W1
Jones, CT1
Zammit, VA1
Brackett, JC; Finegold, DN; Hoffman, EP; Hoop, RC; Naylor, EW; Strauss, AW; Ziadeh, R1
Hoppel, CL; Minkler, PE1
Chace, DH; Chen, YT; Ding, JH; Iafolla, AK; Kahler, SG; Millington, DS; Roe, CR; Terada, N; Van Hove, JL; Zhang, W1
Bennett, MJ; Duran, M; Penn, D; Santer, R; Schmidt-Sommerfeld, E; Stanley, CA1
Novak, RF; Zangar, RC1
Chace, DH; Hillman, SL; Naylor, EW; Van Hove, JL1
Clayton, PT; Doig, M; Ghafari, S; Johnson, AW; Leonard, JV; Meaney, C; Morris, M; Taylor, C1
Mroczkowska, JE; Naleçz, KA; Naleçz, MJ; Roux, FS1
Gellerich, FN; Neuhof, C; Opalka, JR; Trumbeckaite, S; Zierz, S1
Adam, BW; Chace, DH; DiPerna, JC; Hannon, WH1
Craighead, HG; Henion, J; Kameoka, J; Zhang, H1
Carpenter, K; Heath, D; Sim, KG; Wilcken, B; Wiley, V1
Appleton, M; Bartlett, K; Craft, A; Morris, A; Pourfarzam, M1
Liobikas, J; Majiene, D; Toleikis, A; Trumbeckaite, S1
Lehotay, DC; LePage, J; Rockman-Greenberg, C; Thompson, JR1
Hsiao, YS; Jogl, G; Tong, L1
de Assis, DR; Dutra-Filho, CS; Ferreira, GC; Latini, A; Maria, RC; Schuck, PF; Wajner, M; Wannmacher, CM; Wyse, AT1
Baumgartner, C; Baumgartner, D1
Comeau, AM; Eaton, RB; Grady, GF; Hsu, HW; Marsden, D; Shih, VE; Strauss, AW; Zytkovicz, TH1
Busch, U; Fingerhut, R; Liebl, B; Maier, EM; Muntau, AC; Nennstiel-Ratzel, U; Olgemöller, B; Pongratz, J; Roscher, AA; Röschinger, W1
Adams, DJ; Arnold, GL; Caggana, M; Devincentis, E; Erbe, R; Galvin-Parton, PA; Kronn, D; Levy, PA; Mofidi, S; Nichols, M; Pellegrino, JE; Saavedra-Matiz, CA; Wasserstein, M1
Chace, DH; De Jesús, VR; Hannon, WH; Lim, TH; Mei, JV1
Besley, G; Dalton, N; Dezateux, C; Downing, M; Green, A; Henderson, M; Khalid, JM; Krywawych, S; Oerton, J; Wilcken, B; Wiley, V1
Boushel, R; Dela, F; Hansen, CN; Kraunsøe, R; Mikines, KJ; Qvortrup, K; Schjerling, P; Støckel, M1
Gavrilov, D; Highsmith, WE; Matern, D; McHugh, D; Oglesbee, D; Raymond, K; Rinaldo, P; Smith, EH; Thomas, C; Tortorelli, S1
Andersen, JL; Dela, F; Hansen, CN; Helge, JW; Hey-Mogensen, M; Larsen, S; Madsbad, S; Stride, N; Worm, D1
Grings, M; Knebel, LA; Leipnitz, G; Moura, AP; Ribeiro, CA; Tonin, AM; Wajner, M; Zanatta, Â1
Dela, F; Gustafsson, F; Hey-Mogensen, M; Køber, L; Larsen, S; Lund, JT; Sander, K; Stride, N1
Meadows, JA; Wargo, MJ1
Alexandre-Gouabau, MC; Antignac, JP; Courant, F; Darmaun, D; Küster, A; Le Gall, G; Moyon, T; Tea, I1
Moretti, S; Naccarato, A; Sindona, G; Tagarelli, A1
Donzelli, M; Haschke, M; Krähenbühl, S; Morand, R1
Danielsen, JH; Dela, F; Dybboe, R; Helge, JW; Larsen, S; Skaaby, S; Søgaard, D; Søndergård, SD; Vigelsoe, A1
Hankemeier, T; Lindenburg, PW; Oedit, A; Schoonen, JW; van Duinen, V; Vulto, P1
Bramwell, RC; Donato, AJ; Enz, RA; Hart, CR; Hazra, S; Henson, GD; Layec, G; Lesniewski, LA; Morgan, RG; Reihl, KD; Richardson, RS; Trinity, JD; Walker, AE1
Hagar, A; Hall, PL; Wittenauer, A1
Jung, S; Kim, M; Lee, JH; Lee, SH1
Bernstone, L; Hall, SK; Lawson, AJ1
Burgess, SL; Horscroft, JA; Hu, Y; Murray, AJ1
Andrew, B; Bezuidenhout, N; George, S; Maarman, G; Madlala, HP; Mendham, A; Ojuka, E; Osiki, PO1
Aukrust, P; Berge, RK; Damås, JK; Flo, TH; Landrø, L; Svardal, A; Ueland, T; Waagsbø, B; Øktedalen, O1
Reiser, G; Schönfeld, P1
Hoppel, CL; Ingalls, ST; Minkler, PE; Stoll, MSK1
Al Rijjal, D; Batchuluun, B; Bhattacharjee, A; Burdett, E; Eversley, JA; Gunderson, EP; Liu, Y; Mohan, H; Prentice, KJ; Wheeler, MB1
Jo, G; Oh, K; Seo, WK; Shin, MJ1
Bosch, AM; de Vries, M; Derks, TGJ; Gozalbo, ER; Jager, EA; Kuijpers, MM; Mulder, MF; Schielen, PCJI; van Spronsen, FJ; Visser, G; Waterham, HR; Williams, M1
Li, Y; Liu, Y; Song, J; Xu, J; Yang, Y; Zhu, R1
Kim, DH; Kim, TH; Lee, A; Park, J; Shin, Y1
Bovet, C; Gray, PC; Kruithof-de Julio, M; Minoli, M; Ng, CKY; Piscuoglio, S; Spahn, M; Thalmann, GN; Wehrhan, A; Zoni, E1
Canfield, MA; Freedenberg, D; Hua, F; Hunt, P; Jackson, JF; Langlois, PH; Lee, R; Mandell, DJ; Reilly, B; Ruktanonchai, DJ; Rutenberg, GW; Villanacci, JF1
Gornbein, JA; Koos, BJ1
Li, HY; Li, YY; Mu, K; Sun, XC; Xu, J1

Reviews

1 review(s) available for carnitine and octanoylcarnitine

ArticleYear
Measurement of β-oxidation capacity of biological samples by respirometry: a review of principles and substrates.
    American journal of physiology. Endocrinology and metabolism, 2016, 05-01, Volume: 310, Issue:9

    Topics: Acyl Coenzyme A; Carnitine; Coenzyme A; Electrodes; Fatty Acids; Feedback, Physiological; Humans; Malates; Mitochondria; Oxidation-Reduction; Oxidative Phosphorylation; Oxygen Consumption; Palmitoylcarnitine

2016

Trials

1 trial(s) available for carnitine and octanoylcarnitine

ArticleYear
Screening for medium chain acyl-CoA dehydrogenase deficiency using electrospray ionisation tandem mass spectrometry.
    Archives of disease in childhood, 1998, Volume: 79, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adolescent; Age Distribution; Aging; Carnitine; Child; Child, Preschool; Fasting; Female; Heterozygote; Humans; Hypoglycemia; Infant; Infant, Newborn; Male; Mass Screening; Mass Spectrometry; Neonatal Screening; Reference Values; Reye Syndrome

1998

Other Studies

77 other study(ies) available for carnitine and octanoylcarnitine

ArticleYear
[14C]palmitate uptake in isolated rat liver mitochondria: effects of fasting, diabetes mellitus, and inhibitors of carnitine acyltransferase.
    Journal of lipid research, 1978, Volume: 19, Issue:6

    Topics: Acyltransferases; Adenosine Triphosphate; Animals; Caprylates; Carnitine; Carnitine Acyltransferases; Coenzyme A; Diabetes Mellitus, Experimental; Diabetic Ketoacidosis; Male; Mitochondria, Liver; Palmitates; Palmitic Acids; Rats; Starvation

1978
Some aspects of fatty acid oxidation in isolated fat-cell mitochondria from rat.
    The Biochemical journal, 1975, Volume: 152, Issue:3

    Topics: Adenosine Triphosphate; Adipose Tissue; Animals; Bicarbonates; Caprylates; Carbonyl Cyanide p-Trifluoromethoxyphenylhydrazone; Carnitine; Carnitine O-Palmitoyltransferase; Citrate (si)-Synthase; Coenzyme A; DNA; Fatty Acids; Glutamate Dehydrogenase; In Vitro Techniques; Malates; Male; Mitochondria; Mitochondria, Liver; Oxidation-Reduction; Oxygen Consumption; Pyruvates; Rats; Starvation

1975
Direct gas chromatographic assay of urinary medium-chain fatty acylcarnitines by their thermal decomposition.
    Clinica chimica acta; international journal of clinical chemistry, 1992, Feb-14, Volume: 205, Issue:3

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Chromatography, Gas; Chromatography, High Pressure Liquid; Fatty Acid Desaturases; Hot Temperature; Humans

1992
Urinary medium-chain acylcarnitines in medium-chain acyl-CoA dehydrogenase deficiency, medium-chain triglyceride feeding and valproic acid therapy: sensitivity and specificity of the radioisotopic exchange/high performance liquid chromatography method.
    Pediatric research, 1992, Volume: 31, Issue:6

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adolescent; Carnitine; Child; Child, Preschool; Chromatography, High Pressure Liquid; Humans; Infant; Lipid Metabolism, Inborn Errors; Sensitivity and Specificity; Triglycerides; Valproic Acid

1992
Short-term effects of triiodothyronine on exogenous and de novo synthesized fatty acids in rat hepatocytes.
    Biochemistry international, 1991, Volume: 25, Issue:5

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Cells, Cultured; Fatty Acids; Lipids; Liver; Oxidation-Reduction; Palmitic Acid; Palmitic Acids; Phospholipids; Rats; Triiodothyronine

1991
Protein binding of L-carnitine family components.
    European journal of drug metabolism and pharmacokinetics, 1991, Volume: Spec No 3

    Topics: Acetylcarnitine; Animals; Blood Proteins; Carnitine; Chromatography, Gel; Dogs; Humans; Palmitoylcarnitine; Protein Binding; Rats; Serum Albumin; Ultrafiltration

1991
One-step facile synthesis of radioactive acyl-CoA and acylcarnitines using rat liver outer mitochondrial membrane as the enzyme source.
    Biochemical Society transactions, 1991, Volume: 19, Issue:2

    Topics: Acyl Coenzyme A; Animals; Caprylates; Carbon Radioisotopes; Carnitine; Intracellular Membranes; Isotope Labeling; Mitochondria, Liver; Palmitic Acid; Palmitic Acids; Rats; Submitochondrial Particles

1991
Synthesis and purification of radioactive fatty acylcarnitines of high specific activity.
    Analytical biochemistry, 1991, Aug-15, Volume: 197, Issue:1

    Topics: 1-Propanol; Carnitine; Chemistry Techniques, Analytical; Chromatography, Ion Exchange; Heptanes; Hydrochloric Acid; Palmitoylcarnitine; Tritium

1991
Medium-chain acyl-CoA dehydrogenase deficiency.
    The New England journal of medicine, 1989, May-04, Volume: 320, Issue:18

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; Humans; Mass Spectrometry

1989
Metabolism of octanoyl- and palmitoylcarnitine by intact rat hepatocytes.
    Biochimica et biophysica acta, 1989, Jun-08, Volume: 1003, Issue:2

    Topics: Animals; Carnitine; In Vitro Techniques; Ketone Bodies; Liver; Male; Palmitoylcarnitine; Rats; Rats, Inbred Strains

1989
Quantitation of urinary carnitine esters in a patient with medium-chain acyl-coenzyme A dehydrogenase deficiency: effect of metabolic state and L-carnitine therapy.
    The Journal of pediatrics, 1989, Volume: 115, Issue:4

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase; Carnitine; Child; Child, Preschool; Chromatography, High Pressure Liquid; Fasting; Fatty Acid Desaturases; Humans; Infant; Male

1989
Post-mortem recognition of inherited metabolic disorders from specific acylcarnitines in tissue in cases of sudden infant death.
    Lancet (London, England), 1987, Feb-28, Volume: 1, Issue:8531

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Fatty Acid Desaturases; Female; Humans; Infant; Infant, Newborn; Male; Metabolism, Inborn Errors; Sudden Infant Death

1987
Valproylcarnitine: a novel drug metabolite identified by fast atom bombardment and thermospray liquid chromatography-mass spectrometry.
    Clinica chimica acta; international journal of clinical chemistry, 1985, Jan-15, Volume: 145, Issue:1

    Topics: Adolescent; Carnitine; Child; Child, Preschool; Chromatography, High Pressure Liquid; Humans; Isomerism; Male; Mass Spectrometry; Seizures; Valproic Acid

1985
Recognition of medium-chain acyl-CoA dehydrogenase deficiency in asymptomatic siblings of children dying of sudden infant death or Reye-like syndromes.
    The Journal of pediatrics, 1986, Volume: 108, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adult; Breast Feeding; Carnitine; Child, Preschool; Female; Humans; Infant; Mass Spectrometry; Reye Syndrome; Risk; Sudden Infant Death

1986
Diagnostic and therapeutic implications of medium-chain acylcarnitines in the medium-chain acyl-coA dehydrogenase deficiency.
    Pediatric research, 1985, Volume: 19, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; Dicarboxylic Acids; Female; Gas Chromatography-Mass Spectrometry; Humans; Infant; Male; Reye Syndrome

1985
Octanoic acidemia and octanoylcarnitine excretion with dicarboxylic aciduria due to defective oxidation of medium-chain fatty acids.
    The Journal of pediatrics, 1985, Volume: 107, Issue:3

    Topics: Acyl-CoA Dehydrogenases; Caprylates; Carnitine; Cells, Cultured; Decanoic Acids; Dicarboxylic Acids; Fatty Acids; Fatty Acids, Nonesterified; Female; Fibroblasts; Humans; Infant; Lipid Metabolism, Inborn Errors; Male; Oxidation-Reduction

1985
Octanoylglucuronide excretion in patients with a defective oxidation of medium-chain fatty acids.
    Clinica chimica acta; international journal of clinical chemistry, 1985, Nov-15, Volume: 152, Issue:3

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Caprylates; Carnitine; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Hydrogen-Ion Concentration; Lipid Metabolism, Inborn Errors

1985
Studies with (plus)--octanoylcarnitine in experimental diabetic ketoacidosis.
    Diabetes, 1974, Volume: 23, Issue:6

    Topics: Administration, Oral; Animals; Blood Glucose; Caproates; Caprylates; Carnitine; Depression, Chemical; Diabetes Mellitus, Experimental; Diabetic Ketoacidosis; Esters; Hemolysis; In Vitro Techniques; Injections, Intravenous; Insulin; Ketone Bodies; Liver; Male; Stimulation, Chemical

1974
Urinary excretion of l-carnitine and acylcarnitines by patients with disorders of organic acid metabolism: evidence for secondary insufficiency of l-carnitine.
    Pediatric research, 1984, Volume: 18, Issue:12

    Topics: Acyl-CoA Dehydrogenases; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Carnitine; Child; Child, Preschool; Female; Hemiterpenes; Humans; Infant; Infant, Newborn; Lysine; Male; Methylmalonic Acid; Middle Aged; Pentanoic Acids; Propionates

1984
L-carnitine: therapeutic strategy for metabolic encephalopathy.
    Brain research, 1984, Sep-17, Volume: 310, Issue:1

    Topics: Animals; Biological Transport, Active; Brain Diseases; Caprylates; Carnitine; Choroid Plexus; In Vitro Techniques; Metabolic Diseases; Mitochondria; Rabbits

1984
Effects of (+)-octanoylcarnitine on deoxyribonucleic acid synthesis in regenerating rabbit liver.
    Clinical science (London, England : 1979), 1982, Volume: 62, Issue:3

    Topics: Animals; Caprylates; Carnitine; Depression, Chemical; DNA; Hepatectomy; Liver; Liver Regeneration; Male; Rabbits; Thymidine

1982
Changes in predominant energy substrate after hepatectomy.
    Life sciences, 1981, Jan-19, Volume: 28, Issue:3

    Topics: Adenine Nucleotides; Animals; Caprylates; Carnitine; Energy Metabolism; Fatty Acids, Nonesterified; Glucose; Hepatectomy; Ketone Bodies; Male; Mitochondria, Liver; Phosphorylation; Rabbits; Sodium Fluoride; Time Factors

1981
Phosphoenolpyruvate synthesis by fetal guinea-pig liver mitochondria.
    Biochimica et biophysica acta, 1981, Jan-21, Volume: 672, Issue:2

    Topics: Animals; Animals, Newborn; Caprylates; Carnitine; Guinea Pigs; Ketoglutaric Acids; Malates; Mitochondria, Liver; Palmitoylcarnitine; Phosphoenolpyruvate; Succinates

1981
Differences in predominant energy substrate in relation to the resected hepatic mass in the phase immediately after hepatectomy.
    The Journal of laboratory and clinical medicine, 1981, Volume: 97, Issue:6

    Topics: Animals; Caprylates; Carnitine; Energy Metabolism; Fatty Acids; Fatty Acids, Nonesterified; Glucose; Hepatectomy; Liver Regeneration; Male; Rabbits

1981
The effects of energetic steady state, pyruvate concentration, and octanoyl-(--)-carnitine on the relative rates of carboxylation and decarboxylation of pyruvate by rat liver mitochondria.
    The Journal of biological chemistry, 1981, Aug-25, Volume: 256, Issue:16

    Topics: Adenosine Diphosphate; Adenosine Triphosphate; Animals; Caprylates; Carnitine; Citric Acid Cycle; Kinetics; Mitochondria, Liver; Pyruvates; Pyruvic Acid; Rats

1981
Is there a gloxylate cycle in the liver of the fetal guinea pig?
    Biochemical and biophysical research communications, 1980, Jul-31, Volume: 95, Issue:2

    Topics: Animals; Caprylates; Carnitine; Citric Acid Cycle; Female; Fetus; Glucose; Glyoxylates; Guinea Pigs; Isocitrates; Liver; Oxygen Consumption; Palmitic Acids; Perfusion; Pregnancy

1980
The effect of glucagon treatment and starvation of virgin and lactating rats on the rates of oxidation of octanoyl-L-carnitine and octanoate by isolated liver mitochondria.
    The Biochemical journal, 1980, Aug-15, Volume: 190, Issue:2

    Topics: Animals; Antimetabolites; Caprylates; Carnitine; Female; Glucagon; In Vitro Techniques; Kinetics; Lactation; Mitochondria, Liver; Oxidation-Reduction; Oxygen Consumption; Pregnancy; Rats; Starvation

1980
Medium chain acyl-CoA dehydrogenase deficiency in Pennsylvania: neonatal screening shows high incidence and unexpected mutation frequencies.
    Pediatric research, 1995, Volume: 37, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Amino Acid Sequence; Base Sequence; Carnitine; Cohort Studies; DNA; Female; Gene Deletion; Genetic Testing; Heterozygote; Humans; Incidence; Infant, Newborn; Male; Molecular Sequence Data; Mutation; Pennsylvania; Prospective Studies

1995
Quantification of free carnitine, individual short- and medium-chain acylcarnitines, and total carnitine in plasma by high-performance liquid chromatography.
    Analytical biochemistry, 1993, Aug-01, Volume: 212, Issue:2

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Acylation; Carnitine; Chromatography, High Pressure Liquid; Humans; Methylmalonic Acid; Molecular Weight; Propionates; Reference Standards; Reproducibility of Results

1993
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: diagnosis by acylcarnitine analysis in blood.
    American journal of human genetics, 1993, Volume: 52, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; DNA Mutational Analysis; Female; Heterozygote; Homozygote; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mass Spectrometry; Predictive Value of Tests; Reproducibility of Results

1993
Detection of inborn errors of fatty acid oxidation from acylcarnitine analysis of plasma and blood spots with the radioisotopic exchange-high-performance liquid chromatographic method.
    The Journal of pediatrics, 1993, Volume: 122, Issue:5 Pt 1

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Chromatography, High Pressure Liquid; Fatty Acid Desaturases; Humans; Lipid Metabolism, Inborn Errors

1993
Effects of fatty acids and ketone bodies on cytochromes P450 2B, 4A, and 2E1 expression in primary cultured rat hepatocytes.
    Archives of biochemistry and biophysics, 1997, Jan-15, Volume: 337, Issue:2

    Topics: 3-Hydroxybutyric Acid; Acetoacetates; Animals; Carnitine; Cells, Cultured; Cytochrome P-450 CYP2B1; Cytochrome P-450 CYP2E1; Cytochrome P-450 CYP4A; Cytochrome P-450 Enzyme System; Decanoic Acids; Enzyme Inhibitors; Fatty Acids; Fatty Acids, Unsaturated; Glycerol; Hydroxybutyrates; Ketone Bodies; Liver; Mixed Function Oxygenases; Palmitates; Pyruvic Acid; Rats; RNA, Messenger; Triazoles

1997
Rapid diagnosis of MCAD deficiency: quantitative analysis of octanoylcarnitine and other acylcarnitines in newborn blood spots by tandem mass spectrometry.
    Clinical chemistry, 1997, Volume: 43, Issue:11

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Butanols; Carnitine; Humans; Infant, Newborn; Mass Spectrometry; Prospective Studies; Radioisotope Dilution Technique; Reproducibility of Results; Time Factors

1997
Blood-brain barrier controls carnitine level in the brain: a study on a model system with RBE4 cells.
    Biochemical and biophysical research communications, 2000, Jan-07, Volume: 267, Issue:1

    Topics: Acetylcarnitine; Animals; Betaine; Blood-Brain Barrier; Brain; Carnitine; Cell Line; Cell Membrane; Cerebrovascular Circulation; Endothelium, Vascular; Kinetics; Microcirculation; Models, Biological; Rats

2000
Different sensitivity of rabbit heart and skeletal muscle to endotoxin-induced impairment of mitochondrial function.
    European journal of biochemistry, 2001, Volume: 268, Issue:5

    Topics: Animals; Carnitine; Cell Respiration; Electron Transport; Female; Heart; In Vitro Techniques; Lipopolysaccharides; Male; Mitochondria, Muscle; Muscle Fibers, Skeletal; Muscle, Skeletal; Myocardium; Organ Specificity; Perfusion; Pyruvic Acid; Rabbits; Saponins; Sepsis; Specimen Handling; Succinic Acid; Time Factors; Vascular Resistance

2001
Errors caused by the use of D,L-octanoylcarnitine for blood-spot calibrators.
    Clinical chemistry, 2001, Volume: 47, Issue:4

    Topics: Blood Specimen Collection; Calibration; Carnitine; Humans; Indicators and Reagents; Infant, Newborn; Mass Spectrometry; Paper; Stereoisomerism

2001
A polymeric microfluidic chip for CE/MS determination of small molecules.
    Analytical chemistry, 2001, May-01, Volume: 73, Issue:9

    Topics: Acetylcarnitine; Carnitine; Electrophoresis, Capillary; Microscopy, Electron, Scanning; Molecular Structure; Polymers; Spectrometry, Mass, Electrospray Ionization

2001
Evaluation of newborn screening for medium chain acyl-CoA dehydrogenase deficiency in 275 000 babies.
    Archives of disease in childhood. Fetal and neonatal edition, 2001, Volume: 85, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Australian Capital Territory; Carnitine; Humans; Infant, Newborn; Mass Spectrometry; Mutation; Neonatal Screening; New South Wales; Prospective Studies; Retrospective Studies

2001
Neonatal screening for medium-chain acyl-CoA dehydrogenase deficiency.
    Lancet (London, England), 2001, Sep-29, Volume: 358, Issue:9287

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; Humans; Infant, Newborn; Neonatal Screening; Retrospective Studies; United Kingdom

2001
Relevance of fatty acid oxidation in regulation of the outer mitochondrial membrane permeability for ADP.
    FEBS letters, 2001, Dec-07, Volume: 509, Issue:2

    Topics: Adenosine Diphosphate; Animals; Biological Transport; Carnitine; Fatty Acids; Intracellular Membranes; Male; Mitochondria, Heart; Oxidative Phosphorylation; Oxygen Consumption; Palmitoyl Coenzyme A; Palmitoylcarnitine; Permeability; Rats

2001
Blood acylcarnitine levels in normal newborns and heterozygotes for medium-chain acyl-CoA dehydrogenase deficiency: a relationship between genotype and biochemical phenotype?
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Adenine; Carnitine; Diagnosis, Differential; Gene Frequency; Genotype; Guanine; Heterozygote; Humans; Infant, Low Birth Weight; Infant, Newborn; Metabolism, Inborn Errors; Osmolar Concentration; Phenotype

2004
Crystal structure of mouse carnitine octanoyltransferase and molecular determinants of substrate selectivity.
    The Journal of biological chemistry, 2005, Jan-07, Volume: 280, Issue:1

    Topics: Amino Acid Sequence; Animals; Binding Sites; Carnitine; Carnitine Acyltransferases; Catalysis; Crystallography, X-Ray; Mice; Models, Molecular; Molecular Sequence Data; Molecular Structure; Mutagenesis, Site-Directed; Sequence Alignment; Substrate Specificity

2005
Na+, K+ ATPase activity is markedly reduced by cis-4-decenoic acid in synaptic plasma membranes from cerebral cortex of rats.
    Experimental neurology, 2006, Volume: 197, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Animals; Antioxidants; Carnitine; Cerebral Cortex; Enzyme Inhibitors; Fatty Acids, Monounsaturated; Lipid Peroxidation; Luminescent Measurements; Rats; Rats, Wistar; Sodium-Potassium-Exchanging ATPase; Synaptic Membranes; Thiobarbituric Acid Reactive Substances

2006
Biomarker discovery, disease classification, and similarity query processing on high-throughput MS/MS data of inborn errors of metabolism.
    Journal of biomolecular screening, 2006, Volume: 11, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Biomarkers; Carnitine; Case-Control Studies; Humans; Mass Spectrometry; Metabolism, Inborn Errors; Methylmalonic Acid

2006
Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening.
    Pediatrics, 2008, Volume: 121, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Biomarkers; Breast Feeding; Carnitine; Humans; Infant Formula; Infant, Newborn; Neonatal Screening; Point Mutation; Sequence Analysis, DNA

2008
Dissection of biochemical borderline phenotypes in carriers and genetic variants of medium-chain acyl-CoA dehyrogenase deficiency: implications for newborn screening [corrected].
    Clinical genetics, 2009, Volume: 76, Issue:2

    Topics: Carnitine; Case-Control Studies; Heterozygote; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Mutation; Neonatal Screening

2009
Lack of genotype-phenotype correlations and outcome in MCAD deficiency diagnosed by newborn screening in New York State.
    Molecular genetics and metabolism, 2010, Volume: 99, Issue:3

    Topics: Acyl-CoA Dehydrogenases; Carnitine; Fatty Acids; Female; Genotype; Humans; Infant, Newborn; Male; Metabolism, Inborn Errors; Mutation; Neonatal Screening; New York; Phenotype; Prognosis

2010
Comparison of amino acids and acylcarnitines assay methods used in newborn screening assays by tandem mass spectrometry.
    Clinica chimica acta; international journal of clinical chemistry, 2010, May-02, Volume: 411, Issue:9-10

    Topics: Amino Acids; Butanols; Carnitine; Humans; Infant, Newborn; Leucine; Metabolic Diseases; Methionine; Neonatal Screening; Palmitoylcarnitine; Phenylalanine; Quality Control; Tandem Mass Spectrometry

2010
Relationship of octanoylcarnitine concentrations to age at sampling in unaffected newborns screened for medium-chain acyl-CoA dehydrogenase deficiency.
    Clinical chemistry, 2010, Volume: 56, Issue:6

    Topics: Acyl-CoA Dehydrogenase; Age Factors; Amino Acid Metabolism, Inborn Errors; Australia; Carnitine; England; Female; Humans; Infant, Newborn; Male

2010
Mitochondrial respiration in subcutaneous and visceral adipose tissue from patients with morbid obesity.
    The Journal of physiology, 2010, Jun-15, Volume: 588, Issue:Pt 12

    Topics: Adult; Biopsy; Carbonyl Cyanide p-Trifluoromethoxyphenylhydrazone; Carnitine; Cell Respiration; DNA, Mitochondrial; Energy Metabolism; Female; Glutamic Acid; Humans; Intra-Abdominal Fat; Malates; Male; Microscopy, Electron, Transmission; Mitochondria; Obesity, Morbid; Omentum; Oxidative Phosphorylation; Subcutaneous Fat, Abdominal; Succinic Acid; Time Factors; Uncoupling Agents

2010
Allelic diversity in MCAD deficiency: the biochemical classification of 54 variants identified during 5 years of ACADM sequencing.
    Molecular genetics and metabolism, 2010, Volume: 100, Issue:3

    Topics: Acyl-CoA Dehydrogenase; Adolescent; Adult; Alleles; Carnitine; Child; Child, Preschool; DNA Mutational Analysis; Exons; Genetic Association Studies; Genetic Carrier Screening; Genetic Variation; Genotype; Glycine; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Middle Aged; Mutation; Mutation, Missense; Neonatal Screening; Polymorphism, Single Nucleotide; Young Adult

2010
Increased mitochondrial substrate sensitivity in skeletal muscle of patients with type 2 diabetes.
    Diabetologia, 2011, Volume: 54, Issue:6

    Topics: Carnitine; Case-Control Studies; Cell Respiration; Citrate (si)-Synthase; Diabetes Mellitus, Type 2; Glutamic Acid; Humans; Insulin Resistance; Male; Middle Aged; Mitochondria, Muscle; Muscle, Skeletal; Myosin Heavy Chains; Obesity; Substrate Specificity; Succinic Acid; Superoxide Dismutase; Thinness

2011
Disruption of redox homeostasis in cerebral cortex of developing rats by acylcarnitines accumulating in medium-chain acyl-CoA dehydrogenase deficiency.
    International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 2012, Volume: 30, Issue:5

    Topics: Acyl-CoA Dehydrogenase; alpha-Tocopherol; Animals; Carnitine; Cerebral Cortex; Glutathione; Homeostasis; Lipid Metabolism, Inborn Errors; Lipid Peroxidation; Male; Melatonin; Oxidative Stress; Protein Carbonylation; Rats; Rats, Wistar; Statistics, Nonparametric; Thiobarbituric Acid Reactive Substances

2012
Decreased mitochondrial oxidative phosphorylation capacity in the human heart with left ventricular systolic dysfunction.
    European journal of heart failure, 2013, Volume: 15, Issue:2

    Topics: Aged; Biopsy; Carnitine; Creatine; Creatine Kinase, Mitochondrial Form; Energy Metabolism; Fatty Acids; Female; Heart Failure, Systolic; Heart Valve Prosthesis Implantation; Heart-Assist Devices; Humans; Male; Middle Aged; Mitochondria, Heart; Mitochondrial Diseases; Myocardium; Oxidative Phosphorylation; Reference Values; Stroke Volume; Ventricular Dysfunction, Left

2013
Characterization of Pseudomonas aeruginosa growth on O-acylcarnitines and identification of a short-chain acylcarnitine hydrolase.
    Applied and environmental microbiology, 2013, Volume: 79, Issue:11

    Topics: Blotting, Western; Carboxylic Ester Hydrolases; Carnitine; Computational Biology; DNA Primers; Electrophoresis, Polyacrylamide Gel; Enzyme Induction; Genetic Vectors; Hydrolysis; Polymerase Chain Reaction; Pseudomonas aeruginosa; Type C Phospholipases

2013
Maternal and cord blood LC-HRMS metabolomics reveal alterations in energy and polyamine metabolism, and oxidative stress in very-low birth weight infants.
    Journal of proteome research, 2013, Jun-07, Volume: 12, Issue:6

    Topics: Biogenic Polyamines; Carnitine; Female; Fetal Blood; Gestational Age; Glutamic Acid; Glutamine; Humans; Infant; Infant, Newborn; Infant, Premature; Infant, Very Low Birth Weight; Male; Maternal-Fetal Exchange; Pregnancy

2013
Identification and assay of underivatized urinary acylcarnitines by paper spray tandem mass spectrometry.
    Analytical and bioanalytical chemistry, 2013, Volume: 405, Issue:25

    Topics: Adult; Calibration; Carnitine; Humans; Limit of Detection; Paper; Reproducibility of Results; Tandem Mass Spectrometry; Young Adult

2013
Quantification of plasma carnitine and acylcarnitines by high-performance liquid chromatography-tandem mass spectrometry using online solid-phase extraction.
    Analytical and bioanalytical chemistry, 2013, Volume: 405, Issue:27

    Topics: Acetylcarnitine; Carnitine; Chromatography, High Pressure Liquid; Humans; Limit of Detection; Methanol; Palmitoylcarnitine; Reproducibility of Results; Solid Phase Extraction

2013
The effect of high-intensity training on mitochondrial fat oxidation in skeletal muscle and subcutaneous adipose tissue.
    Scandinavian journal of medicine & science in sports, 2015, Volume: 25, Issue:1

    Topics: Adult; Carnitine; Exercise; Female; Humans; Male; Mitochondria; Mitochondria, Muscle; Muscle, Skeletal; Overweight; Oxidative Phosphorylation; Oxygen Consumption; Palmitoylcarnitine; Subcutaneous Fat

2015
Continuous-flow microelectroextraction for enrichment of low abundant compounds.
    Analytical chemistry, 2014, Aug-19, Volume: 86, Issue:16

    Topics: Carnitine; Chromatography, Reverse-Phase; Electrochemical Techniques; Equipment Design; Humans; Laurates; Limit of Detection; Mass Spectrometry; Solid Phase Extraction

2014
The impact of ageing on adipose structure, function and vasculature in the B6D2F1 mouse: evidence of significant multisystem dysfunction.
    The Journal of physiology, 2014, Sep-15, Volume: 592, Issue:18

    Topics: Adipose Tissue; Aging; Animals; Arteries; Body Weight; Carnitine; Glutamic Acid; Malates; Male; Mice; Mitochondria; Neovascularization, Physiologic; Oxidative Stress; Oxygen Consumption; Succinic Acid; Triglycerides; Tyrosine; Vasodilation

2014
Newborn screening for medium chain acyl-CoA dehydrogenase deficiency: performance improvement by monitoring a new ratio.
    Molecular genetics and metabolism, 2014, Volume: 113, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Biomarkers; Carnitine; False Positive Reactions; Heterozygote; Humans; Infant; Infant, Newborn; Infant, Very Low Birth Weight; Lipid Metabolism, Inborn Errors; Neonatal Screening; United States

2014
Association between arterial stiffness and serum L-octanoylcarnitine and lactosylceramide in overweight middle-aged subjects: 3-year follow-up study.
    PloS one, 2015, Volume: 10, Issue:3

    Topics: Adult; Antigens, CD; Blood Pressure; Body Mass Index; Carnitine; Case-Control Studies; Humans; Lactosylceramides; Leucine; Middle Aged; Overweight; Vascular Stiffness

2015
Newborn screening blood spot analysis in the UK: influence of spot size, punch location and haematocrit.
    Journal of medical screening, 2016, Volume: 23, Issue:1

    Topics: Amino Acids; Blood Specimen Collection; Carnitine; Dried Blood Spot Testing; Heel; Hematocrit; Humans; Infant, Newborn; Leucine; Linear Models; Methionine; Neonatal Screening; Phenylalanine; Thyrotropin; Trypsinogen; Tyrosine; United Kingdom

2016
Altered Oxygen Utilisation in Rat Left Ventricle and Soleus after 14 Days, but Not 2 Days, of Environmental Hypoxia.
    PloS one, 2015, Volume: 10, Issue:9

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Energy Metabolism; Gene Expression; Heart Ventricles; Hypoxia; Immunoblotting; Ion Channels; Male; Mitochondria, Muscle; Mitochondrial Proteins; Muscle, Skeletal; Myocardium; Oxidation-Reduction; Oxidative Phosphorylation; Oxygen; Oxygen Consumption; PPAR alpha; Pyruvic Acid; Rats, Wistar; Reverse Transcriptase Polymerase Chain Reaction; Time Factors; Uncoupling Protein 3

2015
Low levels of short- and medium-chain acylcarnitines in HIV-infected patients.
    European journal of clinical investigation, 2016, Volume: 46, Issue:5

    Topics: Adult; Antiretroviral Therapy, Highly Active; Carnitine; Case-Control Studies; Disease Progression; Female; HIV Infections; Humans; Interferon-gamma; Interferon-gamma Release Tests; Longitudinal Studies; Male; Mycobacterium avium Complex; Mycobacterium avium-intracellulare Infection; Tumor Necrosis Factor-alpha

2016
Inhibition of β-oxidation is not a valid therapeutic tool for reducing oxidative stress in conditions of neurodegeneration.
    Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism, 2017, Volume: 37, Issue:3

    Topics: Animals; Brain; Caprylates; Carnitine; Energy Metabolism; Homeostasis; Neurodegenerative Diseases; Oxidation-Reduction; Oxidative Stress; Rats

2017
Correcting false positive medium-chain acyl-CoA dehydrogenase deficiency results from newborn screening; synthesis, purification, and standardization of branched-chain C8 acylcarnitines for use in their selective and accurate absolute quantitation by UHPL
    Molecular genetics and metabolism, 2017, Volume: 120, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Chromatography, High Pressure Liquid; False Positive Reactions; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Neonatal Screening; Sensitivity and Specificity; Tandem Mass Spectrometry

2017
Elevated Medium-Chain Acylcarnitines Are Associated With Gestational Diabetes Mellitus and Early Progression to Type 2 Diabetes and Induce Pancreatic β-Cell Dysfunction.
    Diabetes, 2018, Volume: 67, Issue:5

    Topics: Adult; Animals; Carnitine; Case-Control Studies; Cell Respiration; Diabetes Mellitus, Type 2; Diabetes, Gestational; Disease Progression; Female; Glucose Intolerance; Humans; Insulin; Insulin Secretion; Insulin-Secreting Cells; Islets of Langerhans; Mice; Postpartum Period; Pregnancy

2018
Medium-Chain Acylcarnitines Are Associated With Cardioembolic Stroke and Stroke Recurrence.
    Arteriosclerosis, thrombosis, and vascular biology, 2018, Volume: 38, Issue:9

    Topics: Aged; Biomarkers; Brain Ischemia; Carnitine; Chromatography, Liquid; Female; Humans; Male; Metabolomics; Middle Aged; Recurrence; Stroke; Tandem Mass Spectrometry

2018
A nationwide retrospective observational study of population newborn screening for medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in the Netherlands.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Female; Genotype; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Neonatal Screening; Netherlands; Prevalence; Retrospective Studies

2019
Medium-chain acyl-coenzyme A dehydrogenase deficiency: Six cases in the Chinese population.
    Pediatrics international : official journal of the Japan Pediatric Society, 2019, Volume: 61, Issue:6

    Topics: Acyl-CoA Dehydrogenase; Biomarkers; Carnitine; Child, Preschool; China; Female; Genetic Markers; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mutation; Neonatal Screening

2019
Plasma metabolites as possible biomarkers for diagnosis of breast cancer.
    PloS one, 2019, Volume: 14, Issue:12

    Topics: Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Breast Neoplasms; Carnitine; Case-Control Studies; Chromatography, Liquid; Docosahexaenoic Acids; Female; Humans; Hypoxanthine; Mass Spectrometry; Metabolomics; Middle Aged; Pyrrolidonecarboxylic Acid

2019
Preoperative plasma fatty acid metabolites inform risk of prostate cancer progression and may be used for personalized patient stratification.
    BMC cancer, 2019, Dec-16, Volume: 19, Issue:1

    Topics: Aged; Carnitine; Case-Control Studies; Chromatography, Liquid; Disease Progression; Fatty Acids; Humans; Male; Mass Spectrometry; Metabolomics; Middle Aged; Prognosis; Prostatic Neoplasms; White People

2019
The association between newborn screening analytes as measured on a second screen and childhood autism in a Texas Medicaid population.
    American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics, 2020, Volume: 183, Issue:6

    Topics: Acetylcarnitine; Autism Spectrum Disorder; Autistic Disorder; Biomarkers; Carnitine; Case-Control Studies; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Neonatal Screening; Odds Ratio; Texas

2020
Early pregnancy metabolites predict gestational diabetes mellitus: implications for fetal programming.
    American journal of obstetrics and gynecology, 2021, Volume: 224, Issue:2

    Topics: Adult; Alanine; Arginine; Carnitine; Case-Control Studies; Diabetes, Gestational; Diet Therapy; Dopamine; Early Diagnosis; Epigenesis, Genetic; Female; Fetal Development; Gestational Age; Glucose Tolerance Test; Glucuronides; Humans; Hypoglycemic Agents; Lactones; Lysine; Meglutol; Metabolomics; Neopterin; Orotic Acid; Phenols; Pregnancy; Ribonucleosides; Sulfides

2021
Newborn screening and genetic variation of medium chain acyl-CoA dehydrogenase deficiency in the Chinese population.
    Journal of pediatric endocrinology & metabolism : JPEM, 2022, Oct-26, Volume: 35, Issue:10

    Topics: Acyl-CoA Dehydrogenase; Carnitine; China; Fatty Acids; Genetic Variation; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Neonatal Screening; Retrospective Studies; Tandem Mass Spectrometry

2022