Methods for measurement of fatty acid β-oxidation and acyl-CoA dehydrogenase activity in cultured fibroblasts

  • S. Kølvraa
  • N. Gregersen
Short Communication

Keywords

Public Health Internal Medicine Metabolic Disease Dehydrogenase Activity 

References

  1. Gliemann, J. Insulin-like activity of diluted human serum assayed by an isolated adipose cell method.Diabetes 14 (1965) 643–649Google Scholar
  2. Gregersen, N., Rosleff, F., Kølvraa, S., Hobolth, N., Rasmussen, K. and Lauritzen, R. Non-ketotic C6–C10-dicarboxylic aciduria: Biochemical investigations of two cases.Clin. Chim. Acta 102 (1980) 179–189Google Scholar
  3. Mantagos, S., Genel, M. and Tanaka, K. Ethylmalonic-adipic aciduria:in vivo andin vitro studies indicating deficiency of activities of multiple acyl-CoA dehydrogenases.J. Clin. Invest. 64 (1979) 1580–1589Google Scholar
  4. Naylor, E. W., Mosovich, L. L., Guthrie, R., Evans, J. E. and Tieckelmann, H. Intermittent non-ketotic dicarboxylic aciduria in two siblings with hypoglycemia: an apparent defect in oxidation of fatty acids.J. Inher. Metab. Dis. 3 (1980) 19–24Google Scholar
  5. Rhead, W. J. and Tanaka, K. Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.Proc. Natl. Acad. Sci. U.S.A. 77 (1980) 580–583Google Scholar
  6. Stern, J. R., del Campillo, A. and Raw, I. Enzymes of fatty acid metabolism.J. Biol. Chem. 218 (1956) 971–983Google Scholar
  7. Truscott, R. J. W., Hick, L., Pullin, C., Halpern, B., Wilken, B., Griffiths, H., Silink, M., Kilham, H. and Grunseit, F. Dicarboxylic aciduria: response to fasting.Clin. Chim. Acta 94 (1979) 31–39Google Scholar

Copyright information

© MTP Press Limited 1982

Authors and Affiliations

  • S. Kølvraa
    • 1
  • N. Gregersen
    • 2
  1. 1.Institute for Human GeneticsUniversity of AarhusAarhus CDenmark
  2. 2.Research Laboratory for Metabolic DisordersUniversity Department of Clinical Chemistry, Aarhus KommunehospitalAarhus CDenmark

Personalised recommendations