Oligodendrocyte abnormalities in shiverer mouse mutant are determined in primary chimaeras

Abstract

There is increasing interest in hereditary dysmyelinating mutant mice such as jimpy, quaking, shiverer and twitcher, as animal models for leukodystrophy in man1–3. These mutants are characterized by severe tremor and tonic convulsions. Shiverer is an autosomal recessive mutant which displays abnormal and poor myelination of the central nervous system4–9 and is genetically different from the other dysmyelinating mutants. Despite many reports on the morphological and chemical changes which occur in shiverer mice, the pathogenesis of the abnormal and poor myelin formation is unknown. We have now produced chimaeras by aggregation of eight-cell-stage embryos from wild-type and shiverer mutants. Analysis of the white matter of the chimaera suggests that the oligodendrocyte is responsible for the abnormal and poor myelination in shiverer.