Glucocerebroside storage in normal monocyte cultures.
Glucocerebroside in Gaucher disease is stored in large Gaucher cells of monocyte-macrophage origin. Our aim was to achieve storage of this substrate in monocytes in vitro, in order to mimick the Gaucher cells in vivo. The method described using glucocerebroside-albumin complex, enabled such uptake and storage. The monocytes used changed to large hellical cells. Glucocerebrosidase activity was not affected by the accumulation and storage of the substrate. Although morphological evidence for the accumulation of glucocerebroside within the lysosomes has not yet been obtained, we suggest that these monocytes might represent an in vitro model for the study of Gaucher cells.
D002478:Cells, Cultured; D002554:Cerebrosides / Q000378:metabolism*; D005776:Gaucher Disease / Q000378:metabolism / Q000473:pathology*; D005962:Glucosylceramidase / Q000378:metabolism; D005963:Glucosylceramides / Q000378:metabolism*; D006801:Humans; D008954:Models, Biological; D009000:Monocytes / Q000166:cytology / Q000378:metabolism*
D002554:Cerebrosides; D005963:Glucosylceramides; D005962:Glucosylceramidase