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G-CSFR/CD114 Protein, Antibody, ELISA Kit, cDNA Clone

G-CSFR/CD114 Related Areas

G-CSFR/CD114 Related Pathways

G-CSFR/CD114 Related Product

G-CSFR/CD114 Summary & Protein Information

G-CSFR/CD114 Related Information

G-CSFR/CD114 Background

Gene Summary: The protein encoded by CSF3R gene is the receptor for colony stimulating factor 3, a cytokine that controls the production, differentiation, and function of granulocytes. The encoded protein, which is a member of the family of cytokine receptors, may also function in some cell surface adhesion or recognition processes. Alternatively spliced transcript variants have been described. Mutations in CSF3R gene are a cause of Kostmann syndrome, also known as severe congenital neutropenia. [provided by RefSeq, Aug 2010]
General information above from NCBI
Subunit structure: Homodimer. The dimeric receptor binds two CSF3 molecules.
Domain: The WSXWS motif appears to be necessary for proper protein folding and thereby efficient intracellular transport and cell- surface receptor binding.
The box 1 motif is required for JAK interaction and/or activation.
Subcellular location: Isoform 2: Secreted (Probable).
Cell membrane; Single-pass type I membrane protein.
Tissue specificity: One or several isoforms have been found in myelogenous leukemia cell line KG-1, leukemia U-937 cell line, in bone marrow cells, placenta, and peripheral blood granulocytes. Isoform GCSFR-2 is found only in leukemia U-937 cells. Isoform GCSFR-3 is highly expressed in placenta.
Involvement in disease: Hereditary neutrophilia (NEUTROPHILIA) [MIM:162830]: A form of lifelong, persistent neutrophilia, a condition characterized by an increase in the number of neutrophils in the blood. Note=The disease is caused by mutations affecting the gene represented in this entry.
Sequence similarity: Belongs to the type I cytokine receptor family. Type 2 subfamily.
Contains 5 fibronectin type-III domains.
Contains 1 Ig-like C2-type (immunoglobulin-like) domain.
General information above from UniProt

Granulocyte Colony Stimulating Factor Receptor (G-CSFR), also known as CD114, which belongs to the cytokine receptor superfamily, is a cell surface receptor for colony stimulating factor 3 (CSF3). It is a critical regulator of granulopoiesis. This type I membrane protein has a composite structure consisting of an immunoglobulin(Ig)-like domain, a cytokine receptor-homologous (CRH) domain and three fibronectin type III (FNIII) domains in the extracellular region. Mutations in the G-CSF receptor leading to carboxy-terminal truncation transduce hyperproliferative growth responses, and are implicated in the pathological progression of severe congenital neutropenia (SCN) to acute myelogenous leukemia (AML). Additionally, autocrine/paracrine stimulation of G-CSFR may be important in the biology of solid tumors, including metastasis.

G-CSFR/CD114 Alternative Name

G-CSFR/CD114 Related Studies

  • Kasper B, et al. (1999) Association of src-kinase Lyn and non-src-kinase Syk with the granulocyte colony-stimulating factor receptor (G-CSFR) is not abrogated in neutrophils from severe congenital neutropenia patients with point mutations in the G-CSFR mRNA. Int J Hematol. 70(4): 241-7.
  • Hollenstein U, et al. (2000) Endotoxin down-modulates granulocyte colony-stimulating factor receptor (CD114) on human neutrophils. J Infect Dis. 182(1): 343-6.
  • Kindwall-Keller TL, et al. (2008) Role of the proteasome in modulating native G-CSFR expression. Cytokine. 43(2): 114-23.
  • Beel K, et al. (2009) G-CSF receptor (CSF3R) mutations in X-linked neutropenia evolving to acute myeloid leukemia or myelodysplasia. Haematologica. 94(10): 1449-52.

    G-CSFR/CD114 related areas, pathways, and other information