ICOS (CD278) | Other immune checkpoint proteins | IUPHAR Guide to IMMUNOPHARMACOLOGY

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ICOS (CD278)

Target id: 2939

Nomenclature: ICOS (CD278)

Family: Other immune checkpoint proteins, CD molecules

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

   GtoImmuPdb view: ON :     ICOS (CD278) has curated data in GtoImmuPdb

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human 1 199 2q33.2 ICOS inducible T cell costimulator 3
Mouse 1 200 1 C2; 1 30.6 cM Icos inducible T cell co-stimulator
Rat 1 200 9q32 Icos inducible T-cell co-stimulator
Previous and Unofficial Names
inducible T-cell costimulator | activation-inducible lymphocyte immunomediatory molecule | AILIM | CVID1 | inducible costimulator
Database Links
Ensembl Gene
Entrez Gene
Human Protein Atlas
RefSeq Nucleotide
RefSeq Protein
Natural/Endogenous Ligands
ICOS ligand {Sp: Human}
Clinically-Relevant Mutations and Pathophysiology
Disease:  Common variable immunodeficiency
Description: CVID is a group of genetically heterogeneous primary immunodeficiencies. Whilst a small number of cases can be linked to deficiency in a single gene (see the OMIM links as examples), the cause is likely to be polygenic in the majority of cases. Symptoms vary between patients, but CVID is generally characterised by a failure to produce antibodies (hypogammaglobulinemia) and therefore susceptibility to recurrent infections and inflammation (including autoimmunity).
Disease Ontology: DOID:12177
OMIM: 613495, 607594, 606453, 240500, 613493
Orphanet: ORPHA1572
References:  2,5
General Comments
ICOS belongs to the CD28 and CTLA-4 cell-surface receptor family. It is active as homodimers and is a costimulatory molecule involved in cell-cell signaling, innate immune responses, and regulation of cell proliferation.


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1. Beier KC, Hutloff A, Dittrich AM, Heuck C, Rauch A, Büchner K, Ludewig B, Ochs HD, Mages HW, Kroczek RA. (2000) Induction, binding specificity and function of human ICOS. Eur. J. Immunol., 30 (12): 3707-17. [PMID:11169414]

2. Grimbacher B, Hutloff A, Schlesier M, Glocker E, Warnatz K, Dräger R, Eibel H, Fischer B, Schäffer AA, Mages HW et al.. (2003) Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency. Nat. Immunol., 4 (3): 261-8. [PMID:12577056]

3. Hutloff A, Dittrich AM, Beier KC, Eljaschewitsch B, Kraft R, Anagnostopoulos I, Kroczek RA. (1999) ICOS is an inducible T-cell co-stimulator structurally and functionally related to CD28. Nature, 397 (6716): 263-6. [PMID:9930702]

4. Salomon B, Lenschow DJ, Rhee L, Ashourian N, Singh B, Sharpe A, Bluestone JA. (2000) B7/CD28 costimulation is essential for the homeostasis of the CD4+CD25+ immunoregulatory T cells that control autoimmune diabetes. Immunity, 12 (4): 431-40. [PMID:10795741]

5. Salzer U, Maul-Pavicic A, Cunningham-Rundles C, Urschel S, Belohradsky BH, Litzman J, Holm A, Franco JL, Plebani A, Hammarstrom L et al.. (2004) ICOS deficiency in patients with common variable immunodeficiency. Clin. Immunol., 113 (3): 234-40. [PMID:15507387]

6. Schaefer JS, Klein JR. (2016) Roquin--a multifunctional regulator of immune homeostasis. Genes Immun., 17 (2): 79-84. [PMID:26673963]

7. Vocanson M, Rozieres A, Hennino A, Poyet G, Gaillard V, Renaudineau S, Achachi A, Benetiere J, Kaiserlian D, Dubois B et al.. (2010) Inducible costimulator (ICOS) is a marker for highly suppressive antigen-specific T cells sharing features of TH17/TH1 and regulatory T cells. J. Allergy Clin. Immunol., 126 (2): 280-9, 289.e1-7. [PMID:20624644]

8. Yu D, Tan AH, Hu X, Athanasopoulos V, Simpson N, Silva DG, Hutloff A, Giles KM, Leedman PJ, Lam KP et al.. (2007) Roquin represses autoimmunity by limiting inducible T-cell co-stimulator messenger RNA. Nature, 450 (7167): 299-303. [PMID:18172933]

How to cite this page

Other immune checkpoint proteins: ICOS (CD278). Last modified on 29/01/2018. Accessed on 16/01/2019. IUPHAR/BPS Guide to PHARMACOLOGY, http://guidetoimmunopharmacology.org/GRAC/ObjectDisplayForward?objectId=2939.