Biochemical studies on the leukocytes in chediak-higashi syndrome1

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

doi:10.1159/000207806

Biochemical studies on the leukocytes in chediak-higashi syndrome¹

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

IRCCS pediatrico Burlo Garofolo

Research output: Contribution to journal › Article

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
Pages (from-to)	50-57
Number of pages	8
Journal	Acta Haematologica
Volume	58
Issue number	1
DOIs	https://doi.org/10.1159/000207806
Publication status	Published - 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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10.1159/000207806

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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

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AU - Panizon, F.

PY - 1977

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