High metabolism and subsequent elongation of 3-hydroxyeicosanoyl-CoA in very-long-chain fatty acid deficient PNS of Trembler mice

Neurosci Lett. 1999 Sep 24;273(1):29-32. doi: 10.1016/s0304-3940(99)00600-x.

Abstract

The developmental patterns of the overall fatty acid elongation and of the last two partial activities of microsomal elongase (dehydration and reduction of 3-hydroxyacyl-CoA) were investigated in the PNS of normal and Trembler mice. Unexpectedly, Trembler microsomes synthesized normal C22-CoA amounts from 3-hydroxyeicosanoyl-CoA (3-OHC20-CoA), a C18-CoA elongation intermediate. Hydroxy- acyl-CoA dehydrase and enoyl-CoA reductase activities were found to be higher in the mutant than in the control, whatever the stage of development. Moreover, C20-CoA elongation led to normal C22-CoA and C24-CoA formation in the mutant whereas C20-CoA formation from C18-CoA was always far lower in Trembler than in control. C18-CoA condensing enzyme emerges as the only elongation step involved in the VLCFA deficit evidenced in Trembler PNS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acyl Coenzyme A / metabolism
  • Animals
  • Coenzyme A / metabolism
  • Fatty Acids / metabolism*
  • Hydroxyeicosatetraenoic Acids / metabolism*
  • Malonyl Coenzyme A / metabolism
  • Mice
  • Mice, Neurologic Mutants
  • Microsomes / metabolism
  • Peripheral Nervous System / metabolism*

Substances

  • Acyl Coenzyme A
  • Fatty Acids
  • Hydroxyeicosatetraenoic Acids
  • 3-hydroxy-5,8,11,14-eicosatetraenoic acid
  • Malonyl Coenzyme A
  • Coenzyme A