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- W2115679849 abstract "Certain lysosomal diseases show accretion of residual bodies in peripheral nerves. The presence in peripheral nerves of neuronal processes, Schwann cells of both myelinated and unmyelinated axons, mesenchymal cells and mural cells of the vasculature broadens the spectrum of sites for lysosomal accumulation. We studied the ultrastructure of peripheral nerves in neuronal ceroid lipofuscinosis, type II glycogenosis, GM1-gangliosidosis, late infantile, juvenile and adult type metachromatic leukodystrophies, Krabbe's leukodystrophy, mucopolysaccharidoses, and mucolipidoses. Besides accumulation of disease-specific lysosomal residual bodies in various cell types, mild degenerative features of nerves may occasionally be encountered, probably reflecting metabolic damage of Schwann cells, the cell type most frequently and most severely affected when lysosomal diseases involve the peripheral nerve." @default.
- W2115679849 created "2016-06-24" @default.
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- W2115679849 date "2002-01-01" @default.
- W2115679849 modified "2023-09-30" @default.
- W2115679849 title "Electron Microscopy in Medicine and Biology" @default.
- W2115679849 doi "https://doi.org/10.1016/s0968-4328(00)00076-7" @default.
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