Absence of the major dense line in myelin of the mutant mouse "shiverer".

The myelin of the central nervous system (CNS) of the mutant mouse Shiverer is characterized by the absence of the major dense line (MDL). The intraperiod line, as seen in conventional electron micrographs and in freeze-fractured replicas, appears normal. Peripheral myelin, as seen in ventral and dorsal roots of spinal cord, is unaffected by the mutation. During the period of active myelination, the cytoplasm of most oligodendrocytes (ODs) is packed with electron-lucent vacuoles in continuity with the Golgi apparatus and with bundles of microtubules. It is concluded that a metabolic pathway possibly involving the Golgi apparatus, and contributing to the formation of the MDL is selectively affected in this mutant.