A cholesterol-enriched diet can ameliorate disease in a mouse model of Pelizaeus-Merzbacher disease (PMD), reports a study published this week in Nature Medicine. The findings suggest a potential dietary treatment for individuals with this neurological disease caused by the loss of the protective myelin sheath around neuronal axons.
PMD occurs due to the duplication of a gene called proteolipid protein gene 1, which leads to overexpression of the myelin protein PLP. This overexpressed protein becomes stuck inside the cells, unable to move to the membrane of oligodendrocyte brain cells where it is needed for the proper myelin ensheathment of axons.
Gesine Saher, Klaus Nave and colleagues added cholesterol to oligodendrocyte cells from mice with overexpressed PLP. They found that this led to an increase in PLP trafficking to the cell membrane. They authors also showed that feeding the mice a high-cholesterol diet increased the amount of myelin in their brains and prevented disease progression in the mice.