Cent Eur J Public Health 2004, 12(2):88-90

The Background of beta-Oxidation Disorders in Humans. I. Theory

Kuryl T.1, Debski B.1, Lipko M.1, Martinik K.2
1 Faculty of Veterinary Medicine, Division of Animal Biochemistry, Agricultural University, Warsaw, Poland
2 Pedagogical Faculty, Hradec Králové University, Hradec Králové Czech Republik

Disorders in energetical processes are described from the point of view of energy production and the possibility of substitution of substrates for ATP synthesis. The effects of other diet components such as microelements (Cr3+ ions) and non-digestible oligofructans (inulin and oligofructose) as stimulatory factors on the activity of β-oxidation and cholesterol and triacylglyceroles levels lowering are reported.

Keywords: energy production, β-oxidation, chromium ions, human, rats, broilers

Received: September 23, 2003; Revised: February 4, 2004; Accepted: February 4, 2004; Published: June 1, 2004  Show citation

ACS AIP APA ASA Harvard Chicago Chicago Notes IEEE ISO690 MLA NLM Turabian Vancouver
Kuryl T, Debski B, Lipko M, Martinik K. The Background of beta-Oxidation Disorders in Humans. I. Theory. Cent Eur J Public Health. 2004;12(2):88-90. PubMed PMID: 15242026.
Share...
Download citation
  • BibTeX (.bib)
  • Bookends (.ris)
  • EasyBib (.ris)
  • EndNote (.enw)
  • EndNote 8 (.xml)
  • ISI WoS (.isi)
  • Medlars (.medlars)
  • Mendeley (.ris)
  • MODS (.xml)
  • Papers (.ris)
  • RefWorks (.txt)
  • RefManager (.ris)
  • RIS (.ris)
  • MS Word (.xml)
  • Zotero (.ris)
    • Open full article

    References

    1. Quintana PL, Crespo SP: Diagnostic approach and treatment of inherited mitochondrial fatty acid oxidation disorders.,An Esp Pediatr 2001; 55(6): 524-534. Go to PubMed...
    2. Liu G, Hinch B, BeavisAD: Mechanisms for the transport of α, Ω-dicarboxylates through the mitochondrial inner membrane. J Biol Chem1996; 271 (41): 2538-2544. Go to original source...
    3. Manning NJ, Olpin SE, Pollitt RJ, Webley J: A comparison of [9,10-3 H] palmitic acid and [9,10-3 H] myristic acid for the detection of defects of fatty acids oxidation in intact cultured fibroblasts. J Inher Metab Dis, 1990; 13: 58-68 Go to original source... Go to PubMed...
    4. Olpin SE, Manning NJ, Pollitt RJ, Bonham JR, Downing M, Clark S: The use of [9,10-3 H] palmitate and [9,10-3 H] oleate for detection and diagnosis of medium and long-chain fatty acid oxidation disorders in intact cultured fibroblasts. Adv Exp Med Biol 1999; 466: 321-325. Go to original source...
    5. Kuryl T,Adamowicz M, Dębski B, Bertrandt J, Martinik K: Degradation of [9,10-3 H] myristic acid by lymphocytes. Screening test of inherited disorders of activation, transport and mitochondrial oxidation of fatty acids. Atherosclerosa 2001; 23: 23-26.
    6. Demangre F, Bonnefont J-P, Mitchell G, Nguyen-Hoang N, Pelet A, Rinolds M, Di Donato S, Sandubray JM: Hepatic and muscular presentation of carnitine palmitoyl transferase deficiency: two distinct entieties. Pediatr Res 1988; 24: 308-311. Go to original source... Go to PubMed...
    7. Ijlst L, Mandel H, Oostheim W, Ruiter JPN, Gutman A, Wanders RJA: Molecular basis of hepatic carnitine palmitoyl transferase I deficiency: J Clin Invest 1990; 102: 527-531. Go to original source...
    8. Gibson MK, Lee CF, Kamali V, Sovik O: A coupled assay detecting defect in fibroblasts isoleucine degradation distal to enoyl-CoA hydratase: application to 3-oxothiolase deficiency. Clin Chim Acta 1992; 205: 127-135. Go to original source...
    9. Treem WR, Stanley CA, Finegold DN, Hale DE, Coates PA: Primary carnitine deficiency due to a failure of carnitine transport in kidney, muscle and fibroblasts. N Engl J Med 1988; 319: 1331-1336. Go to original source... Go to PubMed...
    10. Eaton S, Barlett K, Pourfarzam M: Mammalian mitochondrial β-oxidation. Biochem J 1996; 320: 345-347. Go to original source... Go to PubMed...
    11. Elleder M, Kmoch S, Hejtmankowa M, Bouska I: Autopsy and biopsy findings in disorders of mitochondrial β-oxidation of fatty acids. Role of the pathologists in the diagnostic process. Česk Patol 1999; 35(3): 98-105. Go to PubMed...
    12. Horowitz JF: Regulation of lipid mobilization and oxidation during exercise in obesity. Exerc Sport Sci Rev 2001; 29(1): 42-46. Go to original source... Go to PubMed...
    13. Kersten S: Mechanisms of nutritional and hormonal regulation of lipogenesis., EMBO Rep 2001; 2(4): 282-286. Go to original source... Go to PubMed...
    14. Wanders RJA, Vraken M.EJ, den Boer FA: Disorders of mitochondrial fatty acyl-CoA β-oxidation. J Inher Metab Dis 1999; 22: 442-487. Go to original source... Go to PubMed...
    15. Murphy MSR, Kammana VS, Pande SV: A carnitine/acylcarnitine translocase assay applicable to biopsied specimens without requirement mitochondrial isolation. Biochem J 1986; 236: 143-148. Go to original source... Go to PubMed...
    16. Pande SV, Brivet M, SlamaA, Demangre F,Autrant Ch, Sandubray J-M: Carnitine-acylcarnitine translocase deficiency with severe hypoglycemia and auriculo ventricular block. J Clin Invest 1993; 91: 1247-1252. Go to original source... Go to PubMed...
    17. Rasmussen UF, Krustrup P, Bangsbo J, Rasmussen HN: The effect of high-intensity exhaustive exercise studies in isolated mitochondria from human skeletal muscle. Pflugers Arch 2001; 443(2): 180-187. Go to original source... Go to PubMed...
    18. Kuryl T, Lipko M, Dębski B: Metabolism of fatty acids in broiler chicken is affected by chromium. In: Khassanova L, Collery P, Maymard I, Khassanova Z, Etienne JC (eds). Metal ions in biology and medicine. John Libbey Eurotext, vol. 7, 2002. pp. 454-459.
    19. Pettegrew JW, Levine J, McClure RJ: Acetyl-L-carnitine physical-chemical, metabolic, and therapeutic properties: relevance for its mode of action inAlzheimer's disease and geriatric depression. Mol Psychiat 2000; 5(6): 616-632. Go to original source...
    20. Winter S, Birek L, Walker T, Phalin-Roque J, Chandler MJ, Field C, Zorn E: Therapy of metabolic disorders with intravenous (i.v.) access ports and long term intravenous L-carnitine therapy. Southeast Asian J Trop Med Publ Health 1999; 30 (Suppl. 2): 152-153.
    21. Giros M, Ruiz M, Ribes A, Pampols T: The diagnosis of peroxisomal disorders in Spain during the period 1987-1997. Rev Neurol 1999; Suppl. 1: 40-44.
    22. Wanders RJ, Vraken P, Ferdinandusse S, Jansen GA, Waterham HR, van Roermund CW, van Grunsven EG: Peroxisomal fatty acid α- and β-oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal disease. Biochem Soc Trans 2001; 29(2): 250-267. Go to original source...
    23. Rukkwamsuk T, Geelen MJ, Kruip TA, Wensing T: Interrelation of fatty acid composition in adipose tissue, serum and liver of dairy cows during the development of fatty liver postpartum. J Dairy Sci 2000; 83(1): 5259. Go to original source... Go to PubMed...
    24. Kuryl T., Krejpcio Z., Wojciak R., Lipko M., Debski B: The influence of chromium and fructans enriched diet on β-oxidation activity of fatty acids in rat lymphocytes. Mengen- and Spurenelemente, Friedrich Schiller-Universitat Jena 2003 (in press).
    25. Brighenti F, Casiraghi MC, Canzi E, Ferrari A: Effect of consumption of a ready-to-eat breakfast cereal containing inulin on the intestinal milieu and blood lipids in healthy male volunteers. Eur Clin Nutr 1999; 53(9): 726-733. Go to original source...
    26. Letexier D, Diraison F, Beylot M:Addition of inulin to a moderately high-carbohydrate diet reduces hepatic lipogenesis and plasma triacylglycerol concentrations in human. Am J Clin Nutr 2003; 77(3): 559-564. Go to original source... Go to PubMed...

    Return to the content