GAUCHER DISEASE IN MICE INDUCED BY CONDURITOL-B-EPOXIDE - MORPHOLOGIC FEATURES

Abstract

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 s.c. dosage of 100 mg/kg of body wt of conduritol-B-epoxide from 1 day after birth to 28 days. Light microscopically, no Gaucher cells were noted in the viscera, bone marrow and the CNS. Ultrastructurally, the spleen and liver showed irregular granules and fibrils in histiocytes and Kupffer cells during the 2nd-4th wk. In the CNS, the neurons showed fibrils and tubular structures within the cisternae of the endoplasmic reticulum at 3-4 wk. During the 4th wk, they frequently contained well-organized inclusion bodies that were membrane-bound and contained tubular structures that varied from 250-500 A in diameter. These inclusion bodies were similar to Gaucher bodies seen in human cases.