Pathology of visceral organs and bone marrow in an acid sphingomyelinase deficient knock-out mouse line, mimicking human Niemann-Pick disease type A. A light and electron microscopic study.

Abstract

A recently generated aSmase knock-out mouse line develops a lethal storage disease which mimics the neurovisceral form of Niemann-Pick disease in man. In extension to the previously described neuropathological changes, the purpose of this study was to provide a detailed morphological, particularly ultrastructural analysis of the visceral organs of these… (More)

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