ADA deficiency and PNP deficiency

Adenosine deaminase (ADA) is an amino hydrolase that deaminates adenosine to inosine. Absence of ADA results in the accumulation of toxic metabolites including deoxy-ATP interfering with DNA synthesis, causing lymphopenia and SCID. ADA deficiency has been observed in approximately 15% of patients with AR-SCID. Although most patients with ADA deficiency demonstrate severe reduction of T cells, B cells and NK cells, T cells are more sensitive to the toxic metabolites than other lymphocyte sub-populations.

Purine nucleoside phosphorylase (PNP) is an enzyme that converts inosine to hypoxanthine. PNP deficiency results in the accumulation of toxic meta bolites including deoxy-GTP. Generally, the effect of PNP deficiency on lymphocyte number and function is less severe than that seen in ADA deficiency.

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