Abstract
The incorporation of [2-14C]acetate into the lipids of normal and peroxisome-deficient (Zellweger's syndrome) skin fibroblasts was examined. Most of the label was incorporated into triacyglycerol fatty acids in normal as well as Zellweger's syndrome cells. Triacylglycerols and cholesteryl esters in Zellweger's syndrome cells contained increased levels of labelled saturated and monounsaturated very long-chain fatty acids (VLCFA, that is fatty acids with more than 22 carbon atoms), in particular hexacosanoic (26∶0) and hexacosaenoic (26∶1) acids. As traces of labelled VLCFA with up to 32 carbon atoms were detected in triacylglycerols even in control cells it is probable that these fatty acids are formed naturally during the elongation process. Our data suggest that peroxisomes are involved in the chain shortening of the saturated and monounsaturated VLCFA.
Similar content being viewed by others
Abbreviations
- BHT:
-
butylated hydroxytoluene
- BMEM:
-
basal modified Eagle's medium
- VLCFA:
-
very long-chain fatty acids
- TLC:
-
thin-layer chromatography
References
Poulos, A. (1989)Progr. Lipid Res. 28, 35–51.
Schepers, L., Casteels, M., Vamecq, J., Parmentier, G., Van Veldhoven, P.P., and Mannaerts, G.P. (1988)J. Biol. Chem. 263, 2724–2731.
Hajra, A.K., Ghosh, M.K., Weber, K.O., and Datta N.S. (1986) inEnzymes of Lipid Metabolism II (Freysz, L., Dreyfus, H., Massarelli, R., and S. Gatt, eds.) pp. 199–207, Plenum Press, New York.
Singh, H., Usher, S., and Poulos, A. (1989)Arch. Biochem. Biophys. 268 676–686.
Hardeman, D., and van den Bosch, H. (1991)Biochim. Biophys. Acta 1081, 285–292.
Kase, B.F., Prydz, K., Björkhem, I., and Pedersen, J.I. (1986)Biochim. Biophys. Acta 877, 37–42.
Street, J.M., Johnson, D.W., Singh, H., and Poulos, A. (1989)Biochem. J. 260, 647–655.
Street, J.M., Singh, H., and Poulos, A. (1990)Biochem. J. 269, 671–677.
Goldfischer, S., Moore, C.L., Johnson, A.B., Spiro, A.J., Valsamis, M.P., Wisniewski, H.K., Ritch, R.H., Norton, W.T., Rapin, I., and Gartner, L.M. (1973)Science 182, 62–64.
Singh, H., and Poulos, A. (1986)Arch. Biochem. Biophys. 250, 171–179.
Johnson, D.W. (1990)Chem. Phys. Lipids 56, 65–71.
Poulos, A., Bankier, A., Beckman, K., Johnson, D., Robertson, E.F., Sharp, P., Sheffield, L., Singh, H., Usher, S., and Wise, G. (1991)Clin. Genet. 39, 13–25.
Bradford, M.M. (1976)Anal. Biochem. 72, 248–254.
Bligh, B.G., and Dyer, W.J. (1959)Can. J. Biochem. Physiol. 37, 911–917.
James, M.J., and Kandutsch, A.A. (1979)J. Biol. Chem. 254, 8442–8446.
Santos, M.J., Ojeda, J.M., Garrido, J., and Leighton, F. (1985)Proc. Natl. Acad. Sci. USA 82, 6556–6560.
Tsuji, S., Sano, T., Ariga, T., and Miyatake, T. (1981)J. Biochem. 90, 1233–1236.
Tsuji, S., Ohno, T., Miyatake, T., Suzuki, A., and Yamakawa, T. (1984)J. Biochem. 96, 1241–1247.
Christensen, E., Hagve, T.A., and Christophersen, B.O. (1988)Biochim. Biophys. Acta 959, 95–99.
Svennerholm, L., and Ställberg-Stenhagen, S. (1968)J. Lipid Res. 9, 215–225.
Poulos, A., Singh, H., Paton, B., Sharp, P., and Derwas, N. (1986)Clin. Genet. 29, 397–408.
Sharp, P., Johnson, D., and Poulos, A. (1991)J. Neurochem 56, 30–37.
Aveldaño, M., and Sprecher, H. (1987)J. Biol. Chem. 262, 1180–1186.
Poulos, A., Sharp, P., Johnson, D., White, I.G., and Fellenberg, A.J. (1986)Biochem. J. 240, 891–895.
Author information
Authors and Affiliations
About this article
Cite this article
Poulos, A., Usher, S. & Paton, B.C. Fatty acid synthesis from [2-14C]acetate in normal and peroxisome-deficient (Zellweger) fibroblasts. Lipids 28, 97–102 (1993). https://doi.org/10.1007/BF02535771
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02535771