Abnormal lipopigments and lysosomal residual bodies in metachromatic leukodystrophy

Advances in Experimental Medicine and Biology
H H Goebel, H Busch

Abstract

Ultrastructurally, metachromatic leukodystrophy (MLD) is marked by characteristic features such as herringbone, prismatic and tufaceous patterns which are typically encountered within oligodendrocytes of the central nervous system (CNS) and in Schwann cells (PNS). These patterns can be documented in late infantile, juvenile, and adult forms. In the latter, aging of the ailing individual adds another component, the accumulation of lipopigments which are marked by an opaque supposedly lipid droplet and a granular component. While MLD-specific lysosomal residual bodies occur in myelinforming cells, lipopigments accrue in neurons and to a lesser degree in astrocytes. MLD represents a unique example in which these two separate lysosomal storage processes combine to form a wide spectrum of ultrastructurally divergent MLD-lipopigments affecting several cell type in the CNS and PNS. Lipopigments and MLD-specific lysosomal inclusions also assemble in sweat gland epithelial cells again combined within the same residual body and in Schwann cells of non-myelinated axons which may also regularly display lipopigments, contrary to Schwann cells of myelinated axons. Comparative studies on sweat glands in childhood and adult forms confirm the e...Continue Reading

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