ZAP-70, or zeta-associated protein of 70 kDa, is a protein tyrosine kinase that is integral to T-cell receptor (TCR) signaling during T-cell development and activation. It is particularly important for initiating T-cell responses and is predominantly expressed in T cells, which makes it an attractive target for inhibiting pathological T-cell responses in diseases. ZAP-70 deficiency is a genetic disorder within the immune system that results in abnormal T-cell function, preventing T cells from being activated to fight specific infections. This deficiency is a form of severe combined immunodeficiency (SCID) and is inherited in an autosomal recessive pattern. The importance of ZAP-70 in the immune system is underscored by the fact that its absence leads to severe immunodeficiency in both mice and humans. Analyses of the ZAP-70 crystal structure have revealed complex regulatory mechanisms that govern its activity. Furthermore, ZAP-70 plays a role in chronic lymphocytic leukemia and autoimmunity, and its structure, regulation, and function have been extensively studied.
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