CSF2R_HUMAN » Granulocyte-macrophage colony-stimulating factor receptor subunit alpha

CSF2R_HUMAN » Granulocyte-macrophage colony-stimulating factor receptor subunit alpha
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
CSF2R_HUMAN » Granulocyte-macrophage colony-stimulating factor receptor subunit alpha » GM-CSF-R-alpha; GMCSFR-alpha; GMR-alpha; CDw116;
Hydrophobic Thickness 36.8 ± 2.6 Å
Tilt Angle 8 ± 7°
ΔGtransfer -23.8 kcal/mol
ΔGfold -19.6 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 325-350 (324-353)
Pathways

Cytokine-cytokine receptor interaction (KEGG)

Hematopoietic cell lineage (KEGG)

Immune System (Reactome)

Jak-STAT signaling pathway (KEGG)

Pathways in cancer (KEGG)

PDB 3cxe (C=218-300)
OPM none
Complexes

CSF2:CSF2R:IL3RB_HUMAN

Interactions

FATE1, Complex: CSF2R:FATE1

ICAM1, Complex: ICAM1:CSF2R:PTN11:IL3RB:FYN, PubMed

IL3RB, Complex: COF1:KPCB:IL3RB:PLSL:PAXI:CSF2R, PubMed

IL3RB, Complex: CSF2R:CSF2:IL3RB, PDBID: 3cxe

KIT, Complex: CSF2R:KIT, PubMed

SDC2, Complex: CSF2R:SDC2, PubMed

Domains

AA: 123-215, PDBID: 4NKQ, Subunit B, Seq Identity:100%, Interleukin-6 receptor alpha chain, binding

UniProt annotation for CSF2R_HUMAN » Granulocyte-macrophage colony-stimulating factor receptor subunit alpha
FUNCTION: Low affinity receptor for granulocyte-macrophage colony- stimulating factor. Transduces a signal that results in the proliferation, differentiation, and functional activation of hematopoietic cells.

SUBUNIT: Heterodimer of an alpha and a beta subunit. The beta subunit is common to the IL3, IL5 and GM-CSF receptors. The signaling GM-CSF receptor complex is a dodecamer of two head-to- head hexamers of two alpha, two beta, and two ligand subunits.

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: Pulmonary surfactant metabolism dysfunction 4 (SMDP4) OMIM: A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid- Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress. Note=The disease is caused by mutations affecting the gene represented in this entry.

MISCELLANEOUS: The gene coding for this protein is located in the pseudoautosomal region 1 (PAR1) of X and Y chromosomes.

UniProt features for CSF2R_HUMAN » Granulocyte-macrophage colony-stimulating factor receptor subunit alpha
SIGNAL 1 22
CHAIN 23 400 Granulocyte-macrophage colony-stimulating factor receptor subunit alpha.
MOTIF 306 310 WSXWS motif.
MOTIF 355 363 Box 1 motif.
DISULFID 126 136 By similarity.
DISULFID 165 178 By similarity.
Amino Acid Sequence for CSF2R_HUMAN » Granulocyte-macrophage colony-stimulating factor receptor subunit alpha
MLLLVTSLLL CELPHPAFLL IPEKSDLRTV APASSLNVRF DSRTMNLSWD CQENTTFSKC FLTDKKNRVV EPRLSNNECS CTFREICLHE GVTFEVHVNT SQRGFQQKLL YPNSGREGTA AQNFSCFIYN ADLMNCTWAR GPTAPRDVQY FLYIRNSKRR REIRCPYYIQ DSGTHVGCHL DNLSGLTSRN YFLVNGTSRE IGIQFFDSLL DTKKIERFNP PSNVTVRCNT THCLVRWKQP RTYQKLSYLD FQYQLDVHRK NTQPGTENLL INVSGDLENR YNFPSSEPRA KHSVKIRAAD VRILNWSSWS EAIEFGSDDG NLGSVYIYVL LIVGTLVCGI VLGFLFKRFL RIQRLFPPVP QIKDKLNDNH EVEDEIIWEE FTPEEGKGYR EEVLTVKEIT