CSF3R_HUMAN » Granulocyte colony-stimulating factor receptor

CSF3R_HUMAN » Granulocyte colony-stimulating factor receptor
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
CSF3R_HUMAN » Granulocyte colony-stimulating factor receptor » G-CSF receptor; G-CSF-R;
Hydrophobic Thickness 33.2 ± 1.0 Å
Tilt Angle 0 ± 0°
ΔGtransfer -32.1 kcal/mol
ΔGfold -15.5 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 625-648 (621-651)
Pathways

Cytokine-cytokine receptor interaction (KEGG)

Hematopoietic cell lineage (KEGG)

Jak-STAT signaling pathway (KEGG)

Pathways in cancer (KEGG)

PI3K-Akt signaling pathway (KEGG)

PDB 2d9q (B=25-332)
OPM none
Complexes none
Interactions none
Domains

AA: 24-110, PDBID: 2D9Q, Subunit B, Seq Identity:100%, Ig-like C2-type domain

AA: 528-612, PDBID: 3L5H, Subunit A, Seq Identity:30%, Fibronectin type III domain

UniProt annotation for CSF3R_HUMAN » Granulocyte colony-stimulating factor receptor
FUNCTION: Receptor for granulocyte colony-stimulating factor (CSF3), essential for granulocytic maturation. Plays a crucial role in the proliferation, differientation and survival of cells along the neutrophilic lineage. In addition it may function in some adhesion or recognition events at the cell surface.

SUBUNIT: Homodimer. The dimeric receptor binds two CSF3 molecules.

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.

DOMAIN: The WSXWS motif appears to be necessary for proper protein folding and thereby efficient intracellular transport and cell- surface receptor binding.

DOMAIN: The box 1 motif is required for JAK interaction and/or activation.

DISEASE: Hereditary neutrophilia (NEUTROPHILIA) OMIM: A form of lifelong, persistent neutrophilia, a condition characterized by an increase in the number of neutrophils in the blood. by mutations affecting the gene represented in this entry.

MISCELLANEOUS: Mutations in CSF3R acquired in multipotent hematopoietic progenitor cells and resulting in truncated hyper- responsive forms of the receptor, have been identified in most cases of severe congenital neutropenia (SCN). Patients carrying these mutations are at risk for developing myelodysplastic syndromes and/or acute myeloid leukemia. Constitutive mutations leading to hyporesponsive forms of the receptor are responsible for the refractoriness to CSF3 treatment observed in some SCN patients.

UniProt features for CSF3R_HUMAN » Granulocyte colony-stimulating factor receptor
SIGNAL 1 24
CHAIN 25 836 Granulocyte colony-stimulating factor receptor.
DOMAIN 25 117 Ig-like C2-type.
DOMAIN 122 222 Fibronectin type-III 1.
DOMAIN 237 330 Fibronectin type-III 2.
DOMAIN 334 426 Fibronectin type-III 3.
DOMAIN 430 522 Fibronectin type-III 4.
DOMAIN 527 618 Fibronectin type-III 5.
MOTIF 318 322 WSXWS motif.
MOTIF 658 666 Box 1 motif.
DISULFID 26 52
DISULFID 46 101
DISULFID 131 142
DISULFID 167 218
DISULFID 177 186
DISULFID 248 295
DISULFID 266 309
Amino Acid Sequence for CSF3R_HUMAN » Granulocyte colony-stimulating factor receptor
MARLGNCSLT WAALIILLLP GSLEECGHIS VSAPIVHLGD PITASCIIKQ NCSHLDPEPQ ILWRLGAELQ PGGRQQRLSD GTQESIITLP HLNHTQAFLS CCLNWGNSLQ ILDQVELRAG YPPAIPHNLS CLMNLTTSSL ICQWEPGPET HLPTSFTLKS FKSRGNCQTQ GDSILDCVPK DGQSHCCIPR KHLLLYQNMG IWVQAENALG TSMSPQLCLD PMDVVKLEPP MLRTMDPSPE AAPPQAGCLQ LCWEPWQPGL HINQKCELRH KPQRGEASWA LVGPLPLEAL QYELCGLLPA TAYTLQIRCI RWPLPGHWSD WSPSLELRTT ERAPTVRLDT WWRQRQLDPR TVQLFWKPVP LEEDSGRIQG YVVSWRPSGQ AGAILPLCNT TELSCTFHLP SEAQEVALVA YNSAGTSRPT PVVFSESRGP ALTRLHAMAR DPHSLWVGWE PPNPWPQGYV IEWGLGPPSA SNSNKTWRME QNGRATGFLL KENIRPFQLY EIIVTPLYQD TMGPSQHVYA YSQEMAPSHA PELHLKHIGK TWAQLEWVPE PPELGKSPLT HYTIFWTNAQ NQSFSAILNA SSRGFVLHGL EPASLYHIHL MAASQAGATN STVLTLMTLT PEGSELHIIL GLFGLLLLLT CLCGTAWLCC SPNRKNPLWP SVPDPAHSSL GSWVPTIMEE DAFQLPGLGT PPITKLTVLE EDEKKPVPWE SHNSSETCGL PTLVQTYVLQ GDPRAVSTQP QSQSGTSDQV LYGQLLGSPT SPGPGHYLRC DSTQPLLAGL TPSPKSYENL WFQASPLGTL VTPAPSQEDD CVFGPLLNFP LLQGIRVHGM EALGSF