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Insufficiency of ZAP-70: causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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The inadequacy of ZAP-70 (zeta-a-associated protein-70; ZAP-70-zeta-associated protein-70) leads to a disruption in T-lymphocyte activation, which causes defects in signaling systems.
ZAP-70 is important in the interactions of T-lymphocytes and T-cell selection in the thymus. Insufficiency of ZAP-70 leads to defects in T-lymphocyte activation.
In patients with ZAP-70 deficiency in infancy or in early childhood, recurrent infections, such as those that develop with OVID, are noted; However, patients with ZAP-70 deficiency live longer, and the diagnosis can be made only in a few years. Patients may have normal, low or elevated levels of serum immunoglobulins, a normal or increased number of circulating CD4 T-lymphocytes, and no CD8 T-lymphocytes. These CD4 T-lymphocytes do not respond to mitogens or allogeneic cells in vitro and do not form cytotoxic T cells. The activity of natural killers is normal. If bone marrow transplantation is not performed, the disease results in the death of the patient.
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