THE GAUCHER MOUSE: DIFFERENTIAL ACTION OF CONDURITOL B EPOXIDE AND REVERSIBILITY OF ITS EFFECTS |
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| Authors: | M C Stephens A Bernatsky V Burachinsky G Legler J N Kanfer |
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| Institution: | Department of Biochemistry, Faculty of Medicine, University of Manitoba, Winnipeg, Manitoba, R3E OW3, Canada |
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| Abstract: | Abstract— Conduritol B epoxide is an inhibitor of non-mammalian and mammalian β-glucosidase. When injected in mice it produces the biochemical and certain clinical and pathological characteristics of Gaucher disease. An evaluation of the amount required to produce the Gaucher mouse revealed that (1) daily administration of the inhibitor was necessary and (2) lower doses were required to produce accumulation of glucosylceramide in brain than in liver or spleen. It is also demonstrated that reversibility of the effect of conduritol B epoxide can be achieved after it has been injected for a period of 3–4 weeks. |
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