Tomacula in MAG-deficient mice

The pathogenesis of tomacula in mice with a null mutation of the myelin-associated glycoprotein (MAG) gene is not well understood. This study, using a novel teased nerve fiber technique, demonstrates that tomacula in MAG-deficient mice are formed by redundant myelin infoldings and outfoldings in the paranodal regions as early as 4 weeks after birth and increase in size and frequency with age. Although tomacula show degenerative changes with increasing age, there was no significant evidence of demyelination/remyelination. Longitudinal sections of normal teased nerve fibers show early redundant myelin foldings in externally normal paranodal regions. These data and the absence of internodal tomacula support a role for MAG in the maintenance of myelin at the paranodal regions.