Journal
BRITISH JOURNAL OF HAEMATOLOGY
Volume 129, Issue 2, Pages 275-278Publisher
WILEY
DOI: 10.1111/j.1365-2141.2005.05428.x
Keywords
severe congenital neutropenia; myeloid progenitor cells; granulocyte colony-stimulating factor; apoptosis; bcl-2 family members
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Apoptosis is accelerated in the myeloid progenitor cells of patients with severe congenital neutropenia (CN). Granulocyte colony-stimulating factor (G-CSF) increases neutrophil numbers in most CN patients. The effect of G-CSF on apoptosis in CN was analysed by apoptosis rate and expression of anti- and pro-apoptotic factors. G-CSF-treated patients showed higher apoptosis frequency, lower expression of bcl-2 and bcl-xL, but higher expression of bfl-1/A1 and mcl-1. Caspase 9 was highly expressed in patients and controls after G-CSF administration. Thus, G-CSF acts on apoptosis regulation, but additional mechanisms leading to the increase of neutrophil numbers must be assumed.
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