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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

  1. Kameoka J et al Direct association of adenosine deaminase with a T cell activation antigen, CD26. Science 261:466 1992 PMID: 8101391
  2. ADAbase; NOTE=ADA mutation db http://www.uta.fi/imt/bioinfo/ADAbase/
  3. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=ADA
  4. Wikipedia adenosine deaminase entry http://en.wikipedia.org/wiki/Adenosine_deaminase
  5. 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