Adenosine deaminase | Adenosine turnover | IUPHAR Guide to IMMUNOPHARMACOLOGY

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Adenosine deaminase

  Target has curated data in GtoImmuPdb

Target id: 1230

Nomenclature: Adenosine deaminase

Abbreviated Name: ADA

Family: Adenosine turnover

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

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 363 20q13.12 ADA adenosine deaminase
Mouse - 352 2 84.44 cM Ada adenosine deaminase
Rat - 352 3q42 Ada adenosine deaminase
Previous and Unofficial Names
ADA1 | Adenosine aminohydrolase
Database Links
BRENDA
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Enzyme
KEGG Gene
OMIM
Orphanet
UniProtKB
Wikipedia
Enzyme Reaction
EC Number: 3.5.4.4 Adenosine + H2O = inosine + NH3
Rank order of affinity (Human)
2'-deoxyadenosine > adenosine

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Affinity Parameter Reference
EHNA Hs Inhibition 8.8 pKi 1
pKi 8.8 [1]
pentostatin Hs Inhibition 10.8 pIC50 1
pIC50 10.8 [1]
Immunopharmacology Comments
Adenosine deaminase deficiency causes immunodeficiency (ADA deficiency or ADA-SCID). Around 30 known genotypes are associated with this autosomal recessive metabolic disorder. Mitotically active cells such as developing T cells and B cells are susceptible to this deficiency, expaining the resulting pathology.
Immuno Process Associations
Immuno Process:  T cell (activation)
GO Annotations:  Associated to 5 GO processes
GO:0042110 T cell activation IDA
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GO:0002636 positive regulation of germinal center formation IEA
GO:0033089 positive regulation of T cell differentiation in thymus IEA
GO:0046638 positive regulation of alpha-beta T cell differentiation IEA
GO:0070244 negative regulation of thymocyte apoptotic process IEA
Immuno Process:  Cellular signalling
GO Annotations:  Associated to 6 GO processes
GO:0042110 T cell activation IDA
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GO:0002314 germinal center B cell differentiation IEA
GO:0030890 positive regulation of B cell proliferation IEA
GO:0033089 positive regulation of T cell differentiation in thymus IEA
GO:0046638 positive regulation of alpha-beta T cell differentiation IEA
GO:0050862 positive regulation of T cell receptor signaling pathway IEA
Immuno Process:  Inflammation
GO Annotations:  Associated to 3 GO processes, IEA only
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GO:0002314 germinal center B cell differentiation IEA
GO:0002686 negative regulation of leukocyte migration IEA
GO:0050728 negative regulation of inflammatory response IEA
Immuno Process:  B cell (activation)
GO Annotations:  Associated to 4 GO processes, IEA only
click arrow to show/hide IEA associations
GO:0002314 germinal center B cell differentiation IEA
GO:0002636 positive regulation of germinal center formation IEA
GO:0002906 negative regulation of mature B cell apoptotic process IEA
GO:0030890 positive regulation of B cell proliferation IEA
Immuno Process:  Immune regulation
GO Annotations:  Associated to 8 GO processes, IEA only
click arrow to show/hide IEA associations
GO:0002636 positive regulation of germinal center formation IEA
GO:0002686 negative regulation of leukocyte migration IEA
GO:0002906 negative regulation of mature B cell apoptotic process IEA
GO:0030890 positive regulation of B cell proliferation IEA
GO:0033089 positive regulation of T cell differentiation in thymus IEA
GO:0046638 positive regulation of alpha-beta T cell differentiation IEA
GO:0050728 negative regulation of inflammatory response IEA
GO:0050862 positive regulation of T cell receptor signaling pathway IEA
Immuno Process:  Immune system development
GO Annotations:  Associated to 4 GO processes, IEA only
click arrow to show/hide IEA associations
GO:0002314 germinal center B cell differentiation IEA
GO:0033089 positive regulation of T cell differentiation in thymus IEA
GO:0046638 positive regulation of alpha-beta T cell differentiation IEA
GO:0048541 Peyer's patch development IEA
Immuno Process:  Chemotaxis & migration
GO Annotations:  Associated to 3 GO processes, IEA only
click arrow to show/hide IEA associations
GO:0002686 negative regulation of leukocyte migration IEA
GO:0033089 positive regulation of T cell differentiation in thymus IEA
GO:0046638 positive regulation of alpha-beta T cell differentiation IEA
Clinically-Relevant Mutations and Pathophysiology
Disease:  Omenn syndrome
Disease Ontology: DOID:0060010
Orphanet: ORPHA39041
References:  2-3
Disease:  Severe combined immunodeficiency due to adenosine deaminase deficiency
Description: ADA-SCID is an ultra-rare, inherited genetic disorder, caused adenosine deaminase (ADA) deficiency. Affected individuals experience recurrent and potentially life-threatening infections as their immune systems are severely compromised by the build up of toxic metabolites in the absence of ADA activity.
Synonyms: ADA-SCID
Severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, NK cell-negative, due to adenosine deaminase [OMIM: 102700]
Disease Ontology: DOID:5810
OMIM: 102700
Orphanet: ORPHA277
Role: 
Drugs: 
Therapeutic use:  Elapegademase-lvlr can be administered to pediatric and adult patients.

References

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1. Agarwal RP, Spector T, Parks Jr RE. (1977) Tight-binding inhibitors--IV. Inhibition of adenosine deaminases by various inhibitors. Biochem. Pharmacol., 26 (5): 359-67. [PMID:849330]

2. Roifman CM, Zhang J, Atkinson A, Grunebaum E, Mandel K. (2008) Adenosine deaminase deficiency can present with features of Omenn syndrome. J. Allergy Clin. Immunol., 121 (4): 1056-8. [PMID:18243287]

3. Villa A, Notarangelo LD, Roifman CM. (2008) Omenn syndrome: inflammation in leaky severe combined immunodeficiency. J. Allergy Clin. Immunol., 122 (6): 1082-6. [PMID:18992930]

Contributors

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How to cite this page

Detlev Boison.
Adenosine turnover: Adenosine deaminase. Last modified on 09/10/2018. Accessed on 20/05/2019. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetoimmunopharmacology.org/GRAC/ObjectDisplayForward?objectId=1230.