Biochemical studies on the leukocytes in chediak-higashi syndrome1

G. Zabucchi, R. Cramer, M. R. Soranzo, P. Tamaro, F. Panizon

Research output: Contribution to journalArticlepeer-review

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 languageEnglish
Pages (from-to)50-57
Number of pages8
JournalActa Haematologica
Volume58
Issue number1
DOIs
Publication statusPublished - 1977

Keywords

  • Chediak-Higashi syndrome
  • Enzyme release from leukocytes
  • Exo- cytosis lonophore A23187
  • Leukocyte granules
  • Myeloperoxidase

ASJC Scopus subject areas

  • Hematology

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