Role of transverse bands in maintaining paranodal structure and axolemmal domain organization in myelinated nerve fibers: effect on longevity in dysmyelinated mutant mice.

ABSTRACT: The consequences of dysmyelination are poorly understood and vary widely in severity. The shaking mouse, a quaking allele, is characterized by severe central nervous system (CNS) dysmyelination and demyelination, a conspicuous action tremor, and seizures in approximately 25% of animals, but with normal muscle strength and a normal lifespan. In this study we compare this mutant with other dysmyelinated mutants including the ceramide sulfotransferase deficient (CST-/-) mouse, which are more severely affected behaviorally, to determine what might underlie the differences between them with respect to behavior and longevity. Examination of the paranodal junctional region of CNS myelinated fibers shows that "transverse bands," a component of the junction, are present in nearly all shaking parano

SUBMITTER: Mierzwa AJ 

PROVIDER: S-EPMC2879089 | biostudies-literature | 2010 Jul

REPOSITORIES: biostudies-literature