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. |