Investigation of dysmyelinogenesis in caprine beta-mannosidosis: in vitro characterization of oligodendrocytes.
Central nervous system myelin deficiency is a consistent feature of caprine beta-mannosidosis, an autosomal recessive neurovisceral lysosomal storage disease. To investigate the possibility of an intrinsic oligodendrocyte defect in beta-mannosidosis, oligodendrocyte-enriched glial cultures from the cerebral hemisphere white matter of two affected and six control goats were compared with respect to culture yield and morphology. Fewer oligodendrocytes were cultured per gram of white matter from affected animals than from control animals. Galactocerebroside-positive oligodendrocytes from all animals were similar morphologically at all stages of culture by phase contrast and fluorescence microscopy. These findings are consistent with in vivo morphological observations and suggest that differentiated oligodendrocytes from affected animals do not show morphological abnormalities in culture. However, increased numbers of galactocerebroside-negative bipolar cells, which may be glial progenitor cells, were present in cultures from affected animals. This observation suggests the possibility of a defect in differentiation to mature oligodendrocytes, with persistence of the undifferentiated glia during late stages of development.
Boyer
P J
PJ
Department of Pathology, Michigan State University, East Lansing 48824.
Lovell
K L
KL
D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D002452:Cell Count; D002478:Cells, Cultured; D002554:Cerebrosides / Q000378:metabolism*; D005699:Galactosylceramides / Q000378:metabolism*; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006041:Goats; D007150:Immunohistochemistry; D009836:Oligodendroglia / Q000378:metabolism / Q000473:pathology*; D008363:alpha-Mannosidosis / Q000503:physiopathology / Q000662:veterinary*
D002554:Cerebrosides; D005699:Galactosylceramides; D005904:Glial Fibrillary Acidic Protein