Brain Lipid Analysis of a Myelin Deficient Mutant, the “Quaking” Mouse

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Abstract
The “Quaking” mouse is a mutant which has an inherited disease involving the process of myelination in the central nervous system.Fractionation of brain lipids and fatty acid methyl ester analysis of each lipid isolated have been performed. No qualitative abnormalities have been detected, either in ethanolamine, serine and choline phosphoglycerides, or in cholesterol. Proof has now been obtained that the considerable reduction in long chain fatty acids (C24), occurs in cerebrosides, sulphatides (where they are normally major constituents) and in sphingomyelin. Long chain hydroxylated fatty acids are comparatively less diminished than non‐hydroxylated one. This metabolic defect, detected in all the sphingolipids that contain this type of fatty acids could be in favour of a mutation related to fatty acid elongation which is very important at the time of myelin formation.