title: High metabolism and subsequent elongation of 3-hydroxyeicosanoyl-CoA in very-long-chain fatty acid deficient PNS of Trembler mice.
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.
authors.affiliation: Laboratoire de Biogenèse Membranaire, CNRS-UMR 5544, Université Victor Segalen Bordeaux 2, France. francoise.sargueil@biomemb.u-bordeaux2.fr
authors.forename: F
authors.initials: F
authors.lastname: Sargueil
authors.forename: A
authors.initials: A
authors.lastname: Knoll
authors.forename: J
authors.initials: J
authors.lastname: Salles
authors.forename: B
authors.initials: B
authors.lastname: Garbay
authors.forename: C
authors.initials: C
authors.lastname: Cassagne
mesh_terms: D000214:Acyl Coenzyme A / Q000378:metabolism; D000818:Animals; D003065:Coenzyme A / Q000378:metabolism; D005227:Fatty Acids / Q000378:metabolism*; D006893:Hydroxyeicosatetraenoic Acids / Q000378:metabolism*; D008316:Malonyl Coenzyme A / Q000378:metabolism; D051379:Mice; D008818:Mice, Neurologic Mutants; D008861:Microsomes / Q000378:metabolism; D017933:Peripheral Nervous System / Q000378:metabolism*
chemical_list: D000214:Acyl Coenzyme A; D005227:Fatty Acids; D006893:Hydroxyeicosatetraenoic Acids; C069265:3-hydroxy-5,8,11,14-eicosatetraenoic acid; D008316:Malonyl Coenzyme A; D003065:Coenzyme A