Cover: The inherited deficiency of arylsulfatase A causes lysosomal accumulation of the sphingolipid sulfatide and leads to the lysosomal storage disease metachromatic leukodystrophy in humans. The figure shows a kidney section from an arylsulfatase A knockout mouse with massive sulfatide storage (blue) in the medulla and moderate storage in the cortex. As shown in the article, enzyme replacement reduces sulfatide storage in kidney and also improves the nervous system pathology and function. Enzyme replacement might therefore be a promising therapeutic option in this predominantly neurological disease. See article by Matzner et al., pp. 1139-1152.
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