THE GAUCHER MOUSE: DIFFERENTIAL ACTION OF CONDURITOL B EPOXIDE AND REVERSIBILITY OF ITS EFFECTS
- 1 May 1978
- journal article
- research article
- Published by Wiley in Journal of Neurochemistry
- Vol. 30 (5) , 1023-1027
- https://doi.org/10.1111/j.1471-4159.1978.tb12395.x
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.Keywords
This publication has 17 references indexed in Scilit:
- HYDROLYTIC AND TRANSGLUCOLYTIC ACTIVITIES OF A PARTIALLY PURIFIED CALF BRAIN β‐GLUCOSIDASEJournal of Neurochemistry, 1976
- The Gaucher mouseBiochemical and Biophysical Research Communications, 1975
- Synthesis and turnover of lysosomal glycoproteins. Relation to the molecular heterogeneity of the lysosomal enzymesFEBS Letters, 1974
- The bilayer nature of deposits occurring in Gaucher's diseaseArchives of Biochemistry and Biophysics, 1973
- Deficiency of glucosylsphingosine: -Glucosidase in Gaucher diseaseBiochemical and Biophysical Research Communications, 1973
- Labelling of the active centre of a β-glucosidaseBiochimica et Biophysica Acta (BBA) - Enzymology, 1968
- Demonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher's disease.Journal of Clinical Investigation, 1966
- Untersuchungen zum Wirkungsmechanismus glykosidspaltender Enzyme, I. Darstellung und Eigenschaften spezifischer InaktivatorenHoppe-Seyler´s Zeitschrift Für Physiologische Chemie, 1966
- A micro-biuret method for estimating proteinsAnalytical Biochemistry, 1964
- A comparative study of the vascular arrangements in mammalian spleensJournal of Anatomy, 1950