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Details on Person UniProt:P00813 ADA

Class:IdReferenceGeneProduct:49702
_chainChangeLoginitiator methionine:1 added on Fri February 6 2015;chain:2-363 added on Fri February 6 2015;initiator methionine:1 for 49702 removed on Fri Nov 03 2023;initiator methionine: for 49702 added on Fri Nov 03 2023;initiator methionine: for 49702 removed on Fri Aug 15 2025;initiator methionine:1 for 49702 added on Fri Aug 15 2025
_displayNameUniProt:P00813 ADA
_timestamp2026-02-20 21:46:36
chaininitiator methionine:1
chain:2-363
checksum786BC5085CA9AFCB
commentFUNCTION Catalyzes the hydrolytic deamination of adenosine and 2-deoxyadenosine (PubMed:16670267, PubMed:23193172, PubMed:26166670, PubMed:8452534, PubMed:9361033). Plays an important role in purine metabolism and in adenosine homeostasis. Modulates signaling by extracellular adenosine, and so contributes indirectly to cellular signaling events. Acts as a positive regulator of T-cell coactivation, by binding DPP4 (PubMed:20959412). Its interaction with DPP4 regulates lymphocyte-epithelial cell adhesion (PubMed:11772392). Enhances dendritic cell immunogenicity by affecting dendritic cell costimulatory molecule expression and cytokines and chemokines secretion (By similarity). Enhances CD4+ T-cell differentiation and proliferation (PubMed:20959412). Acts as a positive modulator of adenosine receptors ADORA1 and ADORA2A, by enhancing their ligand affinity via conformational change (PubMed:23193172). Stimulates plasminogen activation (PubMed:15016824). Plays a role in male fertility (PubMed:21919946, PubMed:26166670). Plays a protective role in early postimplantation embryonic development (By similarity). Also responsible for the deamination of cordycepin (3'-deoxyadenosine), a fungal natural product that shows antitumor, antibacterial, antifungal, antivirus, and immune regulation properties (PubMed:26038697).CATALYTIC ACTIVITY adenosine + H2O + H(+) = inosine + NH4(+)CATALYTIC ACTIVITY 2'-deoxyadenosine + H2O + H(+) = 2'-deoxyinosine + NH4(+)CATALYTIC ACTIVITY cordycepin + H2O + H(+) = 3'-deoxyinosine + NH4(+)COFACTOR Binds 1 zinc ion per subunit.ACTIVITY REGULATION Inhibited by Cu(2+) and Hg(2+), coformycin, deoxycoformycin (dCF), 2-deoxyadenosine, 6-methylaminopurine riboside, 2-3-iso-propylidene-adenosine, pentostatin, naringin and erythro-9-(2-hydroxy-3-nonyl)adenine (EHNA).BIOPHYSICOCHEMICAL PROPERTIES Interacts with DPP4 (via extracellular domain) (PubMed:10951221, PubMed:14691230, PubMed:15016824, PubMed:7907293, PubMed:8101391). Interacts with PLG (via Kringle 4 domain); the interaction stimulates PLG activation when in complex with DPP4 (PubMed:15016824).INTERACTION Colocalized with DPP4 at the cell surface.TISSUE SPECIFICITY Found in all tissues, occurs in large amounts in T-lymphocytes (PubMed:20959412). Expressed at the time of weaning in gastrointestinal tissues.INDUCTION Up-regulated by hypoxia.POLYMORPHISM There is a common allele, ADA*2, also known as the ADA 2 allozyme. It is associated with the reduced metabolism of adenosine to inosine. It specifically enhances deep sleep and slow-wave activity (SWA) during sleep.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the metallo-dependent hydrolases superfamily. Adenosine and AMP deaminases family.SEQUENCE CAUTION Leiden Open Variation Database (LOVD)ONLINE INFORMATION Adenosine deaminase entry
descriptionrecommendedName: Adenosine deaminase ecNumber evidence="11 17 19 31 34"3.5.4.4 alternativeName: Adenosine aminohydrolase
geneNameADA
ADA1
identifierP00813
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Cell adhesion
Cell junction
Cell membrane
Cytoplasm
Cytoplasmic vesicle
Direct protein sequencing
Disease variant
Hereditary hemolytic anemia
Hydrolase
Lysosome
Membrane
Metal-binding
Nucleotide metabolism
Proteomics identification
Reference proteome
SCID
Zinc
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
nameADA
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8998723] ENSEMBL:ENSG00000196839 ADA [Homo sapiens]
secondaryIdentifierADA_HUMAN
Q53F92
Q6LA59
sequenceLength363
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:49701] ADA [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:9734736] ADA A215T [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:9734741] ADA P297Q [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:9734747] ADA R211C [cytosol] [Homo sapiens]
(referenceSequence)[ReplacedResidue:9734748] L-arginine 211 replaced with L-cysteine
[ReplacedResidue:9734749] L-proline 297 replaced with L-glutamine
[ReplacedResidue:9734750] L-alanine 215 replaced with L-threonine
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No pathways have been reviewed or authored by UniProt:P00813 ADA (49702)