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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

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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)
Immuno Process:  Cellular signalling
Immuno Process:  Inflammation
Immuno Process:  B cell (activation)
Immuno Process:  Immune regulation
Immuno Process:  Immune system development
Immuno Process:  Chemotaxis & migration
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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