Abstract
In order to test the phagocytic function of reticuloendothelial cells in storage diseases, we compared the ingestion and respiratory burst capacities of splenic macrophages (MØ) from a patient with Gaucher's disease to control splenic MØ from patients with Stage IA or IIA Hodgkin's disease. Gaucher (MØ) ingested 14C-labeled E. coli at a rate 22% that of control MØ but bound the E. coli 75% as well as controls. We measured respiratory burst activity in pooled cells by a continuous spectrophotometric assay of superoxide (O2−) production in individual cells by nitro-blue tetrazolium (NBT) slide tests. Both assays utilized phorbol myristate acetate (PMA) a soluble stimulant, or opsonized zymosan (OpZ) particles to initiate the respiratory burst.
Thus Gaucher's disease splenic MØ engorged with cerebroside exhibit a defect not only in ingestion but also in their ability to activate the respiratory burst in response to complement-opsonized particles. However they bind particles nearly as well as controls and produce a normal respiratory burst response to a soluble stimulant.
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Newburger, P., Hansen, S. & Nathan, D. 1051 FED-UP PHAGOCYTES: SPLENIC MACROPHAGE FUNCTION IN GAUCHER'S DISEASE. Pediatr Res 15 (Suppl 4), 618 (1981). https://doi.org/10.1203/00006450-198104001-01077
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DOI: https://doi.org/10.1203/00006450-198104001-01077
