Cerebroside formation in the peripheral nervous system of normal and Trembler mice.
The formation of cerebrosides by the galactosylation of ceramides was investigated in a microsomal fraction prepared from sciatic nerves of normal and Trembler mice. In the control, cerebroside synthesis is observed in the presence of uridine-diphosphate-galactose both from endogenously synthesized [1-14C]stearoyl-sphingosine (C18-ceramide), and from [1-14C]lignoceroyl-sphingosine (C24-ceramide). Cerebroside formation is also demonstrated by studying the galactosylation of exogenous ceramides with UDP[1-14C]-galactose. In the mutant, only trace amounts of labeled cerebrosides are formed from labeled stearoyl-sphingosine, whereas with lignoceroyl-sphingosine, no cerebroside synthesis is detected under conditions allowing their formation in the control. However, a higher rate of synthesis of short acyl chain-glucosyl ceramides is observed in the Trembler samples.
Boiron-Sargueil
F
F
Laboratoire de Biogenèse Membranaire C.N.R.S.-U.R.A. 1811, Université de Bordeaux II, France.
Heape
A
A
Garbay
B
B
Cassagne
C
C
D000818:Animals; D002518:Ceramides / Q000378:metabolism; D002554:Cerebrosides / Q000096:biosynthesis*; D005690:Galactose / Q000378:metabolism; D066298:In Vitro Techniques; D051379:Mice; D008818:Mice, Neurologic Mutants; D008861:Microsomes / Q000378:metabolism; D017933:Peripheral Nervous System / Q000378:metabolism*; D012584:Sciatic Nerve / Q000378:metabolism; D013110:Sphingosine / Q000378:metabolism; D014531:Uridine Diphosphate Galactose / Q000378:metabolism
D002518:Ceramides; D002554:Cerebrosides; D014531:Uridine Diphosphate Galactose; D013110:Sphingosine; D005690:Galactose