Abstract
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.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Antigens, CD / immunology
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Antigens, Differentiation, Myelomonocytic / immunology
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Apoptosis / drug effects
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Biomarkers / analysis
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Case-Control Studies
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Caspase 9
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Caspases / analysis
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Caspases / genetics
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Child, Preschool
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Female
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Fluorescent Antibody Technique
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Gene Expression / drug effects
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Granulocyte Colony-Stimulating Factor / pharmacology
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Humans
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Infant
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Male
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Minor Histocompatibility Antigens
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Myeloid Cell Leukemia Sequence 1 Protein
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Myeloid Progenitor Cells / drug effects
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Myeloid Progenitor Cells / immunology
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Myeloid Progenitor Cells / metabolism*
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Neoplasm Proteins / analysis
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Neoplasm Proteins / genetics
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Neutropenia / congenital*
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Proto-Oncogene Proteins c-bcl-2 / analysis
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Proto-Oncogene Proteins c-bcl-2 / genetics
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Reverse Transcriptase Polymerase Chain Reaction
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Sialic Acid Binding Ig-like Lectin 3
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Stimulation, Chemical
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bcl-X Protein
Substances
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Antigens, CD
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Antigens, Differentiation, Myelomonocytic
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BCL2-related protein A1
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BCL2L1 protein, human
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Biomarkers
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CD33 protein, human
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Minor Histocompatibility Antigens
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Myeloid Cell Leukemia Sequence 1 Protein
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Neoplasm Proteins
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Proto-Oncogene Proteins c-bcl-2
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Sialic Acid Binding Ig-like Lectin 3
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bcl-X Protein
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Granulocyte Colony-Stimulating Factor
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CASP9 protein, human
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Caspase 9
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Caspases