NEUROPATHOLOGY OF MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII

We describe the neuropathology in mucopolysaccharidosis type VII (MPS VII) mice with a recessively inherited deficiency of the lysosomal enz yme beta-glucuronidase. Affected animals have a shortened life span, a re dysmorphic, dwarfed and have clinical evidence of behavioral and me mory deficiencies. Widespread lysosomal distention with glycosaminogly can accumulation affects most viscera. In the central nervous system t here is progressive accumulation of lysosomal storage in neurons, glia and mesenchymal tissue. The morphological character and the amount of lysosomal storage varies among neuronal groups. In the hippocampus, r egional variation in the abundance of lysosomal storage in the MPS VII mice correlates with regional variation in the amount of pb glucuroni dase activity in normal mice, The MPS VII mouse provides a well-define d genetic system for the analysis of the neuropathology of MPS VII and is an attractive model on which to rest the effects of potential ther apies for lysosomal storage disease on the central nervous system.