Brain-specific ceramide synthesis activity: change during brain maturation and in jimpy mouse brain.

The synthesis of ceramide from lignoceric acid was studied in normally developing rat brain and jimpy mouse brain. The developmental pattern of the synthesis of both non-hydroxy and hydroxyceramide from free lignoceric acid in a brain-specific pathway requiring NADPH and cytosolic factors was similar to the synthesis from lignoceroyl CoA and both showed increasing activity during myelination. There were no differences in non-hydroxyceramide synthesis between jimpy and normal littermate control mouse brains by both pathways. HPLC analysis showed the same non-hydroxyceramide level in both types of brains.