Biochemical studies on the leukocytes in Chediak-Higashi syndrome.
Blood leukocytes from a patient with Chediak-Higashi syndrome (CHS) were compared with normal cells for their capacity of extruding (exocytosis) the lysosomal enzyme myeloperoxidase during phagocytosis or after a treatment with the ionophore A23187 and Ca2+. A decreased rate and extent of exocytosis in phagocytizing CHS cells was observed also with the Ca2+ ionophore. This suggests that a defect in Ca2+ mobilization is not responsible for the impaired secretion of granule content. Isolated granules of CHS cells and of leukocytes were treated with the detergent Triton X-100. Since the solubilization of myeloperoxidase from the CHS granules was much lower than from the normal ones, we suggest that the former organelles have a more resistant membrane.
D000001:Calcimycin / Q000494:pharmacology; D002609:Chediak-Higashi Syndrome / Q000097:blood*; D002675:Child, Preschool; D005089:Exocytosis / Q000187:drug effects; D005260:Female; D006801:Humans; D007962:Leukocytes / Q000201:enzymology / Q000378:metabolism* / Q000648:ultrastructure; D009195:Peroxidase / Q000097:blood; D010587:Phagocytosis / Q000187:drug effects; D011092:Polyethylene Glycols / Q000494:pharmacology
D000001:Calcimycin; D011092:Polyethylene Glycols; D009195:Peroxidase