CD118 LIFR (leukemia inhibitory factor receptor α)
Molecule TypeAntigen ExpressionMolecular Weight
Min / Max
Non-lineage Restricted Molecule
Type 1 glycoprotein
Epithelial Cell
Monocyte
Fibroblast
Stem Cell
Liver
Placenta
190 / 190

Expression

CD118 is expressed on adult and embryo epithelial cells, monocytes, fibroblasts, embryonic stem cells, liver and placenta.  Expression is widely outside the immune system but not expressed by resting or activated lymphocytes.  Soluble CD118 is found in serum and rises during pregnancy, in parallel with a drop in circulating LIF levels.



Structure
MOLECULAR FAMILY NAME: Belongs to the cytokine receptor type-1 superfamily.

CD118 is a single-pass type-1 1097 aa glycoprotein.  It contains a 44 aa signal sequence, an 791 aa extracellular domain which contains 2 cytokine receptor homology domains, 1 Ig-like domain, 6 fibronectin type-3 domain with 3 modules, a WSXWS motif which is essential for proper folding and the efficient intracellular transport and cell-surface receptor binding, and 19 potential N-glycosylation sites, a 23 aa transmembrane domain and a 239 aa intracellular cytoplasmic domain which has a box 1 motif essential for signaling throught the JAK system.  CD118 associates noncovalently with CD130 to form the functional LIF receptor

MOLECULAR MASS

POST-TRANSCRIPTIONAL MODIFICATION

Alternative splicing yields 2 different isoforms.  Isoform 1 is a glycoprotein and isoform 2 is a secreted protein.

POST-TRANSLATIONAL MODIFICATION: No information.

Ligands
LIGANDS AND MOLECULES ASSOCIATED WITH CD118

CD118 binds LIF with low affinity and may have inhibitory effects but the CD118/CD130 heterodimer binds LIF with high affinity and is the functional receptor.  Soluble CD118 binding with CD139 may have inhibitory effects.  The CD118/CD130 heterodimer also binds oncostatin M.



Function
CD118 is a leukemia inhibitory factor and is a polyfunctional cytokine that affects cell differentiation, survival, signal transduction and proliferation of a wide variety of cells in the adult and the embryo.  CD118 forms a heterodimer with  CD130 to act as a high affinity receptor LIF.  The CD118/CD130 heterodimer are multifunction cytokines and are members of a family of cytokines that includes components of the receptors for the majority of hematopoietic cytokines and for cytokines that affect other systems, including ciliary neurotrophic factor, growth hormone and prolactin.  The soluble form inhibits the biological activity of CD130 by blocking to receptors on target cells.

BIOCHEMICAL ACTIVITY: No information.

DISEASE RELEVANCE AND FUNCTION OF CD118 IN INTACT ANIMAL

CD118 is widely used for the culture of embryonic stem cells.  Mutations in CD118 are associated with Stuve-Wiedemann sydrome and Schwartz-Jampel type-2 syndrome, a severe autosomal recessive condition belonging to a groups of the bent-bone dysplasias.



Comments
MOLECULAR INTERACTIONS-
PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF CD118: No information.

SUBSTRATES: No information.

ENZYMES WHICH MODIFY OF CD118: No information.

ADDITIONAL INSIGHTS

For further information see Taupin, J. L. et al (2001) J. Biol. Chem. 276: 47975-47981.


Database accession numbers
AnimalPIRSWISSPROTEMGBL/GENBANK
 
HumanEntrezgene 3977P42702
Antibodies

Revised June 25, 2008


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