| First Author | Sargueil F | Year | 1999 |
| Journal | Neurosci Lett | Volume | 273 |
| Issue | 1 | Pages | 29-32 |
| PubMed ID | 10505644 | Mgi Jnum | J:59768 |
| Mgi Id | MGI:1352138 | Doi | 10.1016/s0304-3940(99)00600-x |
| Citation | Sargueil F, et al. (1999) High metabolism and subsequent elongation of 3-hydroxyeicosanoyl-CoA in very-long-chain fatty acid deficient PNS of Trembler mice. Neurosci Lett 273(1):29-32 |
| abstractText | 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. |
