Lipid and Myelin Abnormalities of Brain in the Crinkled Mouse

Abstract

Lipid composition and histology of brain samples from crinkled and non-crinkled mice fed a normal diet were studied. Sulfatides in young and cerebrosides in adults were found to be increased in brains from crinkled mice when compared to their noncrinkled controls. Cholesterol esters, not present at all in the controls, were found in all crinkled brains analyzed. Histological examination of brain showed abnormalities in myelin structures of varying degrees of severity in all brains from crinkled mice. The observation of cholesterol esters in the brains of crinkled mutants supports the view that the myelin disruption found in these mice is secondary to axonal degeneration.