ZAP70 : zeta chain of T cell receptor associated protein kinase 70
Description
The ZAP70 gene provides instructions for making a protein called Zeta-chain-associated protein kinase 70 (ZAP70). This protein plays a crucial role in the immune system, specifically in T cell development and activation. ZAP70 is a tyrosine kinase, an enzyme that adds phosphate groups to other proteins, triggering a cascade of signals that ultimately lead to T cell activation. This activation is essential for mounting an immune response against pathogens and foreign invaders. When T cells encounter their specific antigen, ZAP70 is activated, setting off a chain reaction that results in the production of cytokines, the proliferation of T cells, and the elimination of the target antigen.
Associated Diseases
- Severe Combined Immunodeficiency (SCID): Mutations in ZAP70 can lead to SCID, a life-threatening disorder characterized by a complete lack of functional T cells. This results in severe immune deficiency, making individuals highly susceptible to infections.
- Autoimmune Diseases: In some cases, dysregulation of ZAP70 signaling can contribute to autoimmune diseases. This occurs when the immune system mistakenly attacks the body‘s own tissues.
- Cancer: Some studies suggest that ZAP70 may play a role in the development of certain cancers, particularly those affecting the immune system.
Did you know?
ZAP70 is a relatively large protein, containing over 600 amino acids. It has a unique structure that allows it to bind to the T cell receptor (TCR), a crucial component of the immune system that recognizes and binds to antigens.