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Adenosine deaminase

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Immunopharmacology Ligand target has curated data in GtoImmuPdb

Target id: 1230

Nomenclature: Adenosine deaminase

Abbreviated Name: ADA

Family: Adenosine turnover

Gene and Protein Information Click here for help
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 363 20q13.12 ADA adenosine deaminase
Mouse - 352 2 H3 Ada adenosine deaminase
Rat - 352 3q42 Ada adenosine deaminase
Previous and Unofficial Names Click here for help
ADA1 | Adenosine aminohydrolase
Database Links Click here for help
Alphafold
BRENDA
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Enzyme
KEGG Gene
OMIM
Orphanet
Pharos
UniProtKB
Wikipedia
Enzyme Reaction Click here for help
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 go icon to follow link

Inhibitors
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Value Parameter Reference
EHNA Small molecule or natural product Click here for species-specific activity table Hs Inhibition 8.8 pKi 1
pKi 8.8 [1]
pentostatin Small molecule or natural product Approved drug Primary target of this compound Ligand has a PDB structure 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 8 GO processes
GO:0042110 T cell activation IDA
GO:0045580 regulation of T cell differentiation IMP
GO:0045582 positive regulation of T cell differentiation IMP
GO:0050870 positive regulation of T cell activation IMP
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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 9 GO processes
GO:0042110 T cell activation IDA
GO:0045580 regulation of T cell differentiation IMP
GO:0045582 positive regulation of T cell differentiation IMP
GO:0050870 positive regulation of T cell activation IMP
click arrow to show/hide IEA associations
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
click arrow to show/hide IEA associations
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 11 GO processes, IEA only
GO:0045580 regulation of T cell differentiation IMP
GO:0045582 positive regulation of T cell differentiation IMP
GO:0050870 positive regulation of T cell activation IMP
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 6 GO processes, IEA only
GO:0045580 regulation of T cell differentiation IMP
GO:0045582 positive regulation of T cell differentiation IMP
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 5 GO processes, IEA only
GO:0045582 positive regulation of T cell differentiation IMP
GO:0050870 positive regulation of T cell activation IMP
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 Click here for help
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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