Abstract
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.
Original language | English |
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Pages (from-to) | 50-57 |
Number of pages | 8 |
Journal | Acta Haematologica |
Volume | 58 |
Issue number | 1 |
DOIs | |
Publication status | Published - 1977 |
Keywords
- Chediak-Higashi syndrome
- Enzyme release from leukocytes
- Exo- cytosis lonophore A23187
- Leukocyte granules
- Myeloperoxidase
ASJC Scopus subject areas
- Hematology