Gaucher disease in mice induced by conduritol-B-epoxide: morphologic features

Morphologic changes were studied in mice in which Gaucher disease was induced by the administration of conduritol-B-epoxide. Sixteen newborn Swiss mice received a daily subcutaneous dosage of 100 mg/kg of body weight of conduritol-B-epoxide from one day after birth to 28 days. Light microscopically, no Gaucher cells were noted in the viscera, bone marrow, and the CNS during this study. Ultrastructurally, the spleen and liver showed irregular granules and fibrils in histiocytes and Kupffer cells during the second to fourth week. In the CNS, the neurons showed fibrils and tubular structures within the cisternae of the endoplasmic reticulum at three to four weeks. During the fourth week, they frequently contained well-organized inclusion bodies that were membrane-bound and contained tubular structures that varied from 250 to 500 A in diameter. These inclusion bodies were similar to Gaucher bodies seen in human cases.