Axonal and myelin lesions in β -mannosidosis: Ultrastructural characteristics

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ID: 110515
1970
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Abstract
Ultrastructural changes in central nervous system (CNS) white matter of three goats affected withβ-mannosidosis were analyzed to further define characteristics and pathogenesis of axonal and myelin abnormalities. The variations in myelin association and contents of axonal spheroids were delineated. The occurrence of spheroids in a 96/150-day fetus documented the early development of these axonal lesions. In regions of severe myelin deficits, the presence of apparently normal axons and a reduction in the number of oligodendrocytes were confirmed. Many remaining cells in myelin-deficient regions were characterized by dark, vacuolated cytoplasm. The occurrence of internodes with myelin sheaths adjacent to internodes without myelin sheaths suggested that an axonal defect is not primarily responsible for the absence of myelin sheaths. A mild myelin deficit in the spinal cord was indicated by the presence of unmyelinated axons. Except for occasional mild cytoplasmic vacuolation, the spinal cord glial cells appeared relatively normal. The findings presented here are consistent with the hypothesis that an oligodendrocyte defect, expressed by regional differences, is a major factor in the pathogenesis of myelin deficiency inβ-mannosidosis.
Reference Key
lovell1970actaaxonal Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors K. L. Lovell;M. Z. Jones;K. L. Lovell;M. Z. Jones;
Journal acta neuropathologica
Year 1970
DOI
doi:10.1007/BF00687011
URL
https://link.springer.com/article/10.1007/BF00687011
Keywords
pathology neurosciences

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