Abstract
The dynamic changes of plasma acylcarnitine levels in 1- to 7-y-old children during fasting and after the ingestion of sunflower oil were studied. Glucose, 3-hydroxybutyrate, acetoacetate, FFA, and individual plasma acylcarnitine levels were monitored in both conditions. Fasting experiments lasted for 20 h, and acylcarnitine concentrations were analyzed at 0, 15, and 20 h of fasting. During the fat load, acylcarnitine levels were analyzed at 0, 60, 120, and 180 min. In both tests, a generalized increase of all plasma straight-chain acylcarnitines was observed. Acetylcarnitine contributed the most to the increase of total esterified carnitine. In addition, we demonstrated that the relative increase of each individual acylcarnitine during enhanced fatty acid oxidation is tightly related to its molecular structure and chain length. Fasting as well as the fat load primarily resulted in an increase of unsaturated acylcarnitines. During fasting, C12:1 and C14:1 showed a relatively high increase, whereas after the fat load C16:2 and C14:2, metabolites of linoleic acid (66% of the fat load), were the main acylcarnitines that increased.
Similar content being viewed by others
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
Abbreviations
- CoA:
-
coenzyme A
- LCT:
-
long-chain triglyceride
- KB:
-
ketone bodies
- Glu:
-
glucose
- AcAc:
-
acetoacetate
- 3OHB:
-
3-hydroxybutyrate
- MCT:
-
medium-chain triglyceride
References
McGarry JD, Woeltje KF, Kuwajima M, Foster DW 1989 Regulation of ketogenesis and the renaissance of carnitine palmitoyltransferase. Diabetes Metab Rev 5: 271–284.
Stanley CA, Hale DE 1994 Genetic disorders of mitochondrial fatty acid oxidation. Curr Opin Pediatr 6: 476–481.
Bremer J 1983 Carnitine metabolism, functions. Physiol Rev 63: 1420–1480.
Bieber LL, Emaus R, Valkner K, Farrell S 1982 Possible functions of short-chain and medium-chain carnitine acyltransferases. Fed Proc 41: 2858–2862.
McGarry JD, Robles-Valdes C, Foster DW 1975 Role of carnitine in hepatic ketogenesis. Proc Natl Acad Sci USA 72: 4385–4388.
Paul H, Adibi A 1978 Effect of carnitine on branched-chain amino acid oxidation by liver and skeletal muscle. Am J Physiol 234: E494–E499.
Chalmers RA, Roe CR, Tracey BM, Stacey TE, Hoppel CL, Millington DS 1983 Secondary carnitine insufficiency in disorders of organic acid metabolism: modulation of acyl-CoA/CoA ratios by L-carnitine in vivo. Biochem Soc Trans 11: 724–725.
Millington DS Chace DH Hillman SL Kodo N Terada N Diagnosis of metabolic disease. In: Matsuo T, Caprioli RM, Gross ML, Seyama Y (eds) Biological Mass Spectrometry: Present and Future. John Wiley & Sons, New York, pp 559–579
Frohlich J, Seccombe DW, Jahn P, Dodek P, Hynie I 1978 Effect of fasting on free and esterified carnitine levels in human serum and urine: correlation of serum levels of free fatty acids and 3-hydroxybutyrate. Metabolism 27: 555–561.
Binnert C, Pachiaudi C, Beylot M, Croset M, Cohen R, Riou JP, Laville M 1996 Metabolic fate of an oral long-chain triglyceride load in humans. Am J Physiol 270: E445–E450
Costa CG, Struys EA, Bootsma A, ten Brink HJ, Dorland L, Tavares de Almeida I, Duran M, Jakobs C 1997 Quantitative profiling of plasma acylcarnitines using gas chromatography chemical ionization mass fragmentography. J Lipid Res 38: 173–182.
Dorland L, Horn ME, Struys EA, Costa CG, Ketting D, Duran M, Jakobs C, Tavares de Almeida I, Berger R 1995. Novel FAB-MS of plasma acylcarnitines using single mass spectrometry. 33rd Society for the Study of Inborn Errors of Metabolism (SSIEM) Annual Symposium, Toledo p 137 ( abstr)
Bonnefont JP, Specola NB, Vassault A, Lombes A, Ogier H, de Klerk JBC, Munnich A, Coude M, Paturneau-Jouas M, Saudubray JM 1990 The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states. Eur J Pediatr 150: 80–85.
Connor WE, Lin DS, Colvis C 1996 Differential mobilization of fatty acids from adipose tissue. J Lipid Res 37: 290–298.
Hollenberg CH, Angel A 1963 Relation of fatty acid structure to release and esterification of free fatty acids. Am J Physiol 205: 909–912.
Raclot T, Groscolas R 1993 Differential mobilization of white adipose tissue fatty acids according to chain length, unsaturation, and positional isomerism. J Lipid Res 34: 1515–1526.
Raclot T, Langin D, Lafontan M, Groscolas R 1997 Selective release of human adipose fatty acids according to molecular structure. Biochem J 324: 911–915.
Kler RS, Jackson S, Bartlett K, Bindoff LA, Eaton S, Pourfarzam M, Freerman FE, Goodman SI, Watmough NJ, Turnbull DM 1991 Quantitation of acyl-CoA and acylcarnitine esters accumulated during abnormal mitochondrial fatty acid oxidation. J Biol Chem 266: 22932–22938.
Jackson S, Bartlett K, Land J, Moxon RE, Pollitt RJ, Leonard JV, Turnbull DM 1991 Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatr Res 29: 406–411.
Yang S-Y, He X-Y, Schulz H 1987 Fatty acid oxidation in rat brain is limited by the low activity of 3-ketoacyl-coenzyme A thiolase. J Biol Chem 262: 13027–13032.
Author information
Authors and Affiliations
Additional information
Supported in part by a grant (BD 2589/93-ID) of Junta Nacional de Investigação Científica e Tecnológica-Programa Praxis XXI (Lisbon, Portugal) to Catarina C. G. Costa.
Rights and permissions
About this article
Cite this article
Costa, C., De Almeida, I., Jakobs, C. et al. Dynamic Changes of Plasma Acylcarnitine Levels Induced by Fasting and Sunflower Oil Challenge Test in Children. Pediatr Res 46, 440 (1999). https://doi.org/10.1203/00006450-199910000-00013
Received:
Accepted:
Issue date:
DOI: https://doi.org/10.1203/00006450-199910000-00013
This article is cited by
-
The metabolic footprint of aging in mice
Scientific Reports (2011)
-
Time course of acylcarnitine elevation in neonatal intrahepatic cholestasis caused by citrin deficiency
Journal of Inherited Metabolic Disease (2006)


