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Adenosine Deaminase (ADA) Activity Assay Kit (Fluorometric) (ab204695) is an assay where inosine formed from the breakdown of adenosine is detected via a multi-step reaction, resulting in the formation of an intermediate that reacts with the ADA Probe to generate a fluorescent product that can be easily quantified at Ex/Em = 535/587 nm. The kit measures total activity of Adenosine Deaminase with limit of quantification of 10 μU recombinant Adenosine Deaminase. The range of the assay is from 10-100 μU.
Adenosine Deaminase (ADA, EC 184.108.40.206) is an enzyme that catalyses the conversion of adenosine and 2’-deoxyadenosine to inosine and 2’-deoxyinosine. Adenosine Deaminase is widely distributed in various tissues and cells. There are two isoforms, ADA1 and ADA2. ADA1 is widely expressed in most cells in the body, particularly in lymphocytes and macrophages. It is present in the cytosol, nucleus and has been found associated with DPP4/CD26 in the cell membrane. ADA2 was first found in the spleen but is predominantly found in the plasma and serum. Increased serum ADA levels are found in certain infectious diseases such as tuberculosis and various liver diseases such as acute hepatitis, alcoholic hepatic fibrosis, chronic active hepatitis to name a few. Adenosine Deaminase is also a marker for T-lymphocyte proliferation.
Catalyzes the hydrolytic deamination of adenosine and 2-deoxyadenosine. 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. Its interaction with DPP4 regulates lymphocyte-epithelial cell adhesion.
Found in all tissues, occurs in large amounts in T-lymphocytes and, at the time of weaning, in gastrointestinal tissues.
Involvement in disease
Defects in ADA are the cause of severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-negative due to adenosine deaminase deficiency (ADASCID) [MIM:102700]. SCID refers to a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients with SCID present in infancy with recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development. ADA-SCID is an autosomal recessive form accounting for about 50% of non-X-linked SCIDs. ADA deficiency has been diagnosed in chronically ill teenagers and adults (late or adult onset). Population and newborn screening programs have also identified several healthy individuals with normal immunity who have partial ADA deficiency.
Belongs to the adenosine and AMP deaminases family.
Cell membrane. Cell junction. Cytoplasmic vesicle lumen. Cytoplasm. Colocalized with DPP4 at the cell junction in lymphocyte-epithelial cell adhesion.