Alterations of Cholesterol Synthesis Precursors (7-dehydrocholesterol, 7-dehydrodesmosterol, Desmosterol) in Dysmyelinating Neurological Mutant Mouse (quaking, Shiverer and Trembler) in the PNS and the CNS

In brain, levels of cholesterol, desmosterol and 7-dehydrodesmosterol are reduced in shiverer and quaking, but not in trembler 60-day-old dysmyelinating mutant mice. Very interestingly, 7-dehydrocholesterol is not altered in any mutant. The amount of cholesterol is similar in the different normal control mouse strains and in rat. In contrast, levels of precursors are not the same. In sciatic nerve, cholesterol is slightly reduced in shiverer, reduced 2-fold in quaking, and dramatically reduced in trembler (10-fold). 7-Dehydrocholesterol is affected in all mutants.