immunology allergic disorders
ZAP 70 Deficiency
Introduction
ZAP-70 (zeta-associated protein 70) deficiency is an immunodeficiency disorder involving impaired T-cell activation caused by a signaling defect. (See also Overview of Immunodeficiency Disorders and ZAP-70 deficiency is a Primary Immunodeficiencies that involves ZAP-70 is important in T-cell signaling and in T-cell selection in the thymus. ZAP-70 deficiency causes T-cell activation defects. Patients who have ZAP-70 deficiency present during infancy or early childhood with recurrent infections similar to those in Severe Combined Immunodeficiency (SCID) (SCID); however, they live longer, and the deficiency may not be diagnosed until they are several years old. 