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 …

TA Kuemmel, J Thiele, R Schroeder… - Pathology-Research and …, 1997 - Elsevier
A recently generated aSmase knock-out mouse line develops a lethal storage disease
which mimics the neurovisceral form of Niemann-Pick disease in manIn 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
animals including spleen, liver, intestine, lung, and kidney along with a sequential
histological investigation of the bone marrowOur results showed a progressive lysosomal …