title: Heterogeneous expression pattern of pro- and anti-apoptotic factors in myeloid progenitor cells of patients with severe congenital neutropenia treated with granulocyte colony-stimulating factor.
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.
authors.affiliation: Department of Paediatric Haematology and Oncology, Hannover Medical School, Hannover, Germany.
authors.forename: Gunnar
authors.initials: G
authors.lastname: Cario
authors.forename: Julia
authors.initials: J
authors.lastname: Skokowa
authors.forename: Zheng
authors.initials: Z
authors.lastname: Wang
authors.forename: Vesna
authors.initials: V
authors.lastname: Bucan
authors.forename: Cornelia
authors.initials: C
authors.lastname: Zeidler
authors.forename: Martin
authors.initials: M
authors.lastname: Stanulla
authors.forename: Martin
authors.initials: M
authors.lastname: Schrappe
authors.forename: Karl
authors.initials: K
authors.lastname: Welte
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000276:immunology; D015214:Antigens, Differentiation, Myelomonocytic / Q000276:immunology; D017209:Apoptosis / Q000187:drug effects; D015415:Biomarkers / Q000032:analysis; D016022:Case-Control Studies; D053453:Caspase 9; D020169:Caspases / Q000032:analysis / Q000235:genetics; D002675:Child, Preschool; D005260:Female; D005455:Fluorescent Antibody Technique; D015870:Gene Expression / Q000187:drug effects; D016179:Granulocyte Colony-Stimulating Factor / Q000494:pharmacology; D006801:Humans; D007223:Infant; D008297:Male; D015778:Minor Histocompatibility Antigens; D064549:Myeloid Cell Leukemia Sequence 1 Protein; D023461:Myeloid Progenitor Cells / Q000187:drug effects / Q000276:immunology / Q000378:metabolism*; D009363:Neoplasm Proteins / Q000032:analysis / Q000235:genetics; D009503:Neutropenia / Q000151:congenital*; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000032:analysis / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D063268:Sialic Acid Binding Ig-like Lectin 3; D013268:Stimulation, Chemical; D051020:bcl-X Protein
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; C096816:BCL2-related protein A1; C494811:BCL2L1 protein, human; D015415:Biomarkers; C571163:CD33 protein, human; D015778:Minor Histocompatibility Antigens; D064549:Myeloid Cell Leukemia Sequence 1 Protein; D009363:Neoplasm Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D063268:Sialic Acid Binding Ig-like Lectin 3; D051020:bcl-X Protein; D016179:Granulocyte Colony-Stimulating Factor; C506433:CASP9 protein, human; D053453:Caspase 9; D020169:Caspases