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 | |
| Publication status | Published - 1977 |
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Keywords
- Chediak-Higashi syndrome
- Enzyme release from leukocytes
- Exo- cytosis lonophore A23187
- Leukocyte granules
- Myeloperoxidase
ASJC Scopus subject areas
- Hematology
Cite this
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 journal › Article
}
TY - JOUR
T1 - Biochemical studies on the leukocytes in chediak-higashi syndrome1
AU - Zabucchi, G.
AU - Cramer, R.
AU - Soranzo, M. R.
AU - Tamaro, P.
AU - Panizon, F.
PY - 1977
Y1 - 1977
N2 - 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.
AB - 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.
KW - Chediak-Higashi syndrome
KW - Enzyme release from leukocytes
KW - Exo- cytosis lonophore A23187
KW - Leukocyte granules
KW - Myeloperoxidase
UR - http://www.scopus.com/inward/record.url?scp=0017622973&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0017622973&partnerID=8YFLogxK
U2 - 10.1159/000207806
DO - 10.1159/000207806
M3 - Article
C2 - 199018
AN - SCOPUS:0017622973
VL - 58
SP - 50
EP - 57
JO - Acta Haematologica
JF - Acta Haematologica
SN - 0001-5792
IS - 1
ER -