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The Gaucher mouse.
Authors:J N Kanfer  G Legler  J Sullivan  S S Raghavan  R A Mumford
Institution:1. E.K. Shriver Center at the W.E. Fernald State School, Waltham, MA 02154, USA;2. Institute fur Biochemie der Universitat Koln, Koln, Germany
Abstract:A model of Gauchers disease was produced through the administration of conduritol B-expoxide. Tissue levels of glucosylceramide were elevated in the experimental animals. The activity of β-glucosidase in homogenates of brain, liver, and spleen was reduced 93% in the treated animals. Six other lysosomal hydrolases measured were uneffected.
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