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adenosine deaminase (adenosine aminohydrolase, ADA)
Function:
- deamination of adenosine to inosine
- ADA forms complexes with auxilliary protein, ADA complexing protein-1
- for adenosine deaminases acting on RNA, gene mutations that change guanosine to adenosine may be deaminated to inosine
- protein synthesis elongation reads inosine as guanosine [5]
- extracellular ADA complexes with CD26
Expression:
- expressed in all cells
- highest expression in lymphocytes.
Pathology:
- deficiency causes adenosine deaminase deficiency a form of severe combined immunodeficiency disease (SCID)
- in hereditary hemolytic anemia, the level of this enzyme in erythrocytes increases 50-70 times
Polymorphism:
- common allele, ADA*2, also known as the ADA 2 allozyme associated with diminished metabolism of adenosine to inosine specifically enhances deep sleep slow-wave activity during sleep
Laboratory:
- adenosine deaminase in erythrocytes
- adenosine deaminase in leukocytes
- adenosine deaminase in fibroblasts
- adenosine deaminase in trophoblasts
- adenosine deaminase in chorionic villus
- adenosine deaminase in body fluid
- adenosine deaminase in blood
- adenosine deaminase in dried blood spot
- adenosine deaminase in CSF
- adenosine deaminase in pericardial fluid
- adenosine deaminase in peritoneal fluid
- adenosine deaminase in peritoneal dialysis fluid
- adenosine deaminase in pleural fluid
- adenosine deaminase in serum/plasma
- adenosine deaminase in synovial fluid
Related
adenosine deaminase-like protein (ADAL)
tRNA-specific adenosine deaminase
Specific
adenosine deaminase CECR1; adenosine deaminase 2; cat eye syndrome critical region protein 1 (CECR1, ADA2)
adenosine deaminase complex
General
nucleoside deaminase
Properties
SIZE: entity length = 363 aa
MW = 41 kD
COMPARTMENT: cytoplasm
CELL: most cell types*
plasma
MOTIF: active site
histidine residue {H214}
cysteine residue {C262}
aspartate residue {D295}
aspartate residue {D296}
Database Correlations
OMIM 102700
UniProt P00813
Pfam PF00962
Entrez Gene 100
Kegg hsa/hsa00230
ENZYME 3.5.4.4
References
- Kameoka J et al
Direct association of adenosine deaminase with a T cell
activation antigen, CD26.
Science 261:466 1992
PMID: 8101391
- ADAbase; NOTE=ADA mutation db
http://www.uta.fi/imt/bioinfo/ADAbase/
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=ADA
- Wikipedia adenosine deaminase entry
http://en.wikipedia.org/wiki/Adenosine_deaminase
- Leslie M
Edit (don't kill) the messenger
New treatments aim to counteract mutant genes by fixing the faulty RNA they produce.
Science. 2024. Oct 24.
https://www.science.org/content/article/buoyed-milestone-clinical-result-rna-editing-poised-treat-diseases
Component-of
adenosine deaminase complex