title: Granulocyte colony-stimulating factor receptor signaling in severe congenital neutropenia, chronic neutrophilic leukemia, and related malignancies.
abstract: Granulocyte colony-stimulating factor is a hematopoietic cytokine that stimulates neutrophil production and hematopoietic stem cell mobilization by initiating the dimerization of homodimeric granulocyte colony-stimulating factor receptor. Different mutations of CSF3R have been linked to a unique spectrum of myeloid disorders and related malignancies. Myeloid disorders caused by the CSF3R mutations include severe congenital neutropenia, chronic neutrophilic leukemia, and atypical chronic myeloid leukemia. In this review, we provide an analysis of granulocyte colony-stimulating factor receptor, various mutations, and their roles in the severe congenital neutropenia, chronic neutrophilic leukemia, and malignant transformation, as well as the clinical implications and some perspective on approaches that could expand our knowledge with respect to the normal signaling mechanisms and those associated with mutations in the receptor.
authors: Department of Cancer Biology, University of Cincinnati College of Medicine, Cincinnati, OH.
authors: Pankaj
authors: P
authors: Dwivedi
authors: Department of Cancer Biology, University of Cincinnati College of Medicine, Cincinnati, OH. Electronic address: ken.greis@uc.edu.
authors: Kenneth D
authors: KD
authors: Greis
mesh_terms: D000818:Animals; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000378:metabolism*; D000080984:Congenital Bone Marrow Failure Syndromes; D020022:Genetic Predisposition to Disease; D006801:Humans; D053612:Janus Kinases / Q000378:metabolism; D015470:Leukemia, Myeloid, Acute / Q000209:etiology / Q000378:metabolism; D015467:Leukemia, Neutrophilic, Chronic / Q000209:etiology* / Q000378:metabolism*; D020935:MAP Kinase Signaling System; D009154:Mutation; D009503:Neutropenia / Q000151:congenital* / Q000209:etiology / Q000378:metabolism; D019869:Phosphatidylinositol 3-Kinases / Q000378:metabolism; D011485:Protein Binding; D054730:Protein Interaction Domains and Motifs / Q000235:genetics; D051057:Proto-Oncogene Proteins c-akt / Q000378:metabolism; D016188:Receptors, Granulocyte Colony-Stimulating Factor / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D050791:STAT Transcription Factors / Q000378:metabolism; D015398:Signal Transduction
chemical_list: D053612:Janus Kinases; D019869:Phosphatidylinositol 3-Kinases; D051057:Proto-Oncogene Proteins c-akt; D016188:Receptors, Granulocyte Colony-Stimulating Factor; D050791:STAT Transcription Factors