CD119 IFNGR1, IFNγR(interferon γR), IFNγRα
Molecule TypeAntigen ExpressionMolecular Weight
Min / Max
Non-lineage Restricted Molecule
Type 1 glycoprotein
Monocyte
Macrophage
T Cell
B Cell
NK Cell
Neutrophil
Fibroblast
Epithelial Cell
Endothelium
Tumor Cell
90 / 90
100 / 100

Expression
CD119 is expressed on monocytes, macrophages, T and B cells, NK cells, neutrophils, fibroblasts, epithelial cells, endothelium and a wide range of tumor cells.

Structure
MOLECULAR FAMILY NAME: Belongs to the cytokine type-2 receptor family.

CD119 is a single-pass type-1 489 aa glycoprotein.  It contains a 227 aa extracellular domain which contains 2 Ig-like C2-type domains, 2 fibronectin type-3 domains and has 5 potential N-linked glycosylation sites, a 22 aa transmembrane domains and a 222 aa intracellular cytoplasmic domain.  The functional IFNg consists of a complex formed between CD119 and  IFNg AF-1.  Both CD119 and IFNg AF-1 belong to the class II cytokine receptor family within the Ig gene superfamily.  The WSXWS motif, characteristic of class I cytokine receptors, is absent from both fibronectin type-3 domains in CD119 and IFNg AF-1.

MOLECULAR MASS
Cell Type Unreduced Reduced
90 - 100 kDa

POST-TRANSCRIPTIONAL MODIFICATION: No information.

POST-TRANSLATIONAL MODIFICATION: No information.


Ligands
LIGANDS AND MOLECULES ASSOCIATED WITH CD119

IFNgR binds IFNg with a high affinity of kD = 1 nM-10 pM.  Although CD119 alone can bind IFNg with a high affinity of kD = 50 pM  and does not require the presence of IFNγAF-1 for ligand binding.  The species-specific accessory factor IFNg AF-1, which interacts with the extracellular region of CD119, is required for signal transduction.  In Colo-205 cells, a human adenocarcinoma cell line, the IFNgR receptor is constitutively phosphorylated on Ser and Thr residues and its phosphorylation is enhanced by IFNg or phorbol ester.  


Function
CD119 forms part of the receptor to IFNγ.  Despite being able to bind IFNγ on its own, CD119 must form a complex with IFNγAF-1 for signal transduction to occur.  The CD119 receptorz plays a role in host defense and immunological processes and a role in the initiation and effector phases of immune responses, including macrophage activation, B and T cell differentiation, activation of NK cells and upregulating the expression of MHC Class I and II antigens in several cell types.

BIOCHEMICAL ACTIVITY: No information.

DISEASE RELEVANCE AND FUNCTION OF CD119 IN INTACT ANIMAL

Defects in CD119 are a cause of familial disseminated atypical mycobacterial infection and disseminated BCG infection.  CD119 deficient mice have greater susceptibility to Listeria monocytogenes and vaccine virus but are resistant to endotoxic shock.


Comments
The IFNg AF-1 protein reconstitutes IFNg-induced Class I MHC expression, but not viral resistance, when transfected into heterologous cells together with the human CD119 and HLA-B7 genes.  This suggests that other accessory factors are necessary for IFNg -mediated activities such as antiviral responses.  Despite normal T cell responses, CD119-deficient mice show increased susceptibility to infection by Listeria monocytogenes and vaccinia virus, but are resistant to endotoxic shock.

MOLECULAR INTERACTIONS-
PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF CD119: No information.

SUBSTRATES: No information.

ENZYMES WHICH MODIFY CD119: No information.



Database accession numbers
AnimalPIRSWISSPROTEMGBL/GENBANK
 
HumanEntrezgene 3459P15260
MouseA34368P15261M26711
Antibodies
BB1E2   View Reactivity

Revised June 25, 2008


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