Biochemical studies on the leukocytes in chediak-higashi syndrome1

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

Research output: Contribution to journalArticle

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

Fingerprint

Chediak-Higashi Syndrome
Leukocytes
Ionophores
Exocytosis
Peroxidase
Calcimycin
Octoxynol
Phagocytosis
Organelles
Detergents
Membranes
Enzymes

Keywords

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

ASJC Scopus subject areas

  • Hematology

Cite this

Zabucchi, G., Cramer, R., Soranzo, M. R., Tamaro, P., & Panizon, F. (1977). Biochemical studies on the leukocytes in chediak-higashi syndrome1. Acta Haematologica, 58(1), 50-57. https://doi.org/10.1159/000207806

Biochemical studies on the leukocytes in chediak-higashi syndrome1. / Zabucchi, G.; Cramer, R.; Soranzo, M. R.; Tamaro, P.; Panizon, F.

In: Acta Haematologica, Vol. 58, No. 1, 1977, p. 50-57.

Research output: Contribution to journalArticle

Zabucchi, G, Cramer, R, Soranzo, MR, Tamaro, P & Panizon, F 1977, 'Biochemical studies on the leukocytes in chediak-higashi syndrome1', Acta Haematologica, vol. 58, no. 1, pp. 50-57. https://doi.org/10.1159/000207806
Zabucchi, G. ; Cramer, R. ; Soranzo, M. R. ; Tamaro, P. ; Panizon, F. / Biochemical studies on the leukocytes in chediak-higashi syndrome1. In: Acta Haematologica. 1977 ; Vol. 58, No. 1. pp. 50-57.
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