Summary
In metachromatic leucodystrophy abnormal lipids are stored within the lysosomes of the glial cells and phagocytes. The lipid inclusions which are the less structured are the richest in acid phosphatases. Those of which the ultrastructure is prismatic show a very low enzymatic activity and are more similar to residual bodies than to active lysosomes. It appears that the lack in lysosomal arylsulfatase leads to an accumulation of the sulfatide moiety of the stored lipids while the other lipids originating from the myelin breakdown, would be normally catabolized. This leads to a progressive transformation of the ultrastructure of the lysosome contents.
Résumé
Dans la leucodystrophie métachromatique, les lipides anormaux s'accumulent dans les lysosomes des cellules gliales et des phagocytes. Les inclusions lipidiques les moins organisées sont les plus riches en phosphatases acides. Celles dont l'ultrastructure est prismatique ont une activité enzymatique très faible et semblent plus proches de corps résiduels que de lysosomes actifs. Il semble que l'absence d'arylsulfatase lysosomiale induise la persistance des sulfatides accumulés dans les lysosomes alors que les autres lipides provenant de la dégénérescence myélinique, seraient normalement catabolisés. Un remaniement progressif de l'ultrastructure du contenu des lysosomes en est la conséquence.
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Résibois, A. Electron microscopic study of metachromatic leucodystrophy. Acta Neuropathol 13, 149–156 (1969). https://doi.org/10.1007/BF00687027
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DOI: https://doi.org/10.1007/BF00687027