[Biosynthesis of lignoceric acid in two organelles (mitochondria and microsomes) during the development of the brain in normal and pathologic (Quaking and Jimpy) mice].

In microsomes, biosynthesis of lignoceric acid from its direct precursor (behenyl-CoA) is largely increased during myelination. The peak is hardly detectable in Quaking; in Jimpy, the synthesis is nearly absent (3% of normal value); in the adult Quaking, the synthesis is normal. Mitochondria are capable of synthesizing lignoceric acid. This synthesis increases regularly during brain development and is normal in both mutants. Saturated fatty acid analysis is brain mitochondria shows that these organelles contain mainly palmitic and stearic acids. However, very long chains are also detectable. Thus it appears that mitochondria synthesize their own acids. Microsomes synthesize their own acids also and myelin fatty acids. There is no interplay between microsomal and mitochondrial metabolisms.