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Publication : Lack of in vitro ceramide formation in the PNS of trembler mice.

First Author  Boiron-Sargueil F Year  1992
Journal  J Neurochem Volume  59
Issue  2 Pages  652-6
PubMed ID  1629735 Mgi Jnum  J:1428
Mgi Id  MGI:49955 Doi  10.1111/j.1471-4159.1992.tb09419.x
Citation  Boiron-Sargueil F, et al. (1992) Lack of in vitro ceramide formation in the PNS of trembler mice. J Neurochem 59(2):652-6
abstractText  The amidification of sphingosine by acyl donors has been investigated in a microsomal fraction prepared from sciatic nerves of normal and Trembler mice. In the control, a ceramide synthesis is observed in the presence of acyl-CoAs and not with free fatty acids. The synthesis increases as a function of the protein amount and the time and is dependent on acyl-CoA concentration. The level of synthesis is highly similar to that observed in vivo after palmitate injection into the sciatic nerves of normal mice. In the mutant, there is a major abnormality because a weak synthesis (20% of the control) is observed only with high acyl-CoA concentration (greater than 200 microM), whereas in the range of the physiological acyl-CoA concentrations (less than 20 microM), there is no ceramide formation from stearoyl-CoA or lignoceroyl-CoA.
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