Rabbit anti-Human ZAP70 Polyclonal Antibody

Uniprot Id

P43403

Target Species

Human

Target Name

ZAP70

Target Full Name

Tyrosine-protein kinase ZAP-70

Target Function

Tyrosine kinase that plays an essential role in regulation of the adaptive immune response. Regulates motility, adhesion and cytokine expression of mature T-cells, as well as thymocyte development. Contributes also to the development and activation of primary B-lymphocytes. When antigen presenting cells (APC) activate T-cell receptor (TCR), a serie of phosphorylations lead to the recruitment of ZAP70 to the doubly phosphorylated TCR component CD247/CD3Z through ITAM motif at the plasma membrane. This recruitment serves to localization to the stimulated TCR and to relieve its autoinhibited conformation. Release of ZAP70 active conformation is further stabilized by phosphorylation mediated by LCK. Subsequently, ZAP70 phosphorylates at least 2 essential adapter proteins: LAT and LCP2. In turn, a large number of signaling molecules are recruited and ultimately lead to lymphokine production, T-cell proliferation and differentiation. Furthermore, ZAP70 controls cytoskeleton modifications, adhesion and mobility of T-lymphocytes, thus ensuring correct delivery of effectors to the APC. ZAP70 is also required for TCR-CD247/CD3Z internalization and degradation through interaction with the E3 ubiquitin-protein ligase CBL and adapter proteins SLA and SLA2. Thus, ZAP70 regulates both T-cell activation switch on and switch off by modulating TCR expression at the T-cell surface. During thymocyte development, ZAP70 promotes survival and cell-cycle progression of developing thymocytes before positive selection (when cells are still CD4/CD8 double negative). Additionally, ZAP70-dependent signaling pathway may also contribute to primary B-cells formation and activation through B-cell receptor (BCR).

Target Involvement

Immunodeficiency 48 (IMD48); Autoimmune disease, multisystem, infantile-onset, 2 (ADMIO2)

Target Subcellular Location

Cytoplasm. Cell membrane; Peripheral membrane protein.

Target Protein Families

Protein kinase superfamily, Tyr protein kinase family, SYK/ZAP-70 subfamily

Target Tissue Specificity

Expressed in T- and natural killer cells. Also present in early thymocytes and pro/pre B-cells.

Target Research Area

Immunology

Target Synonyms

70 kDa zeta associated protein; 70 kDa zeta-associated protein; EC 2.7.10.2; FLJ17670; FLJ17679; Selective T cell defect; SRK; STD; Syk related tyrosine kinase; Syk-related tyrosine kinase; Truncated ZAP kinase; Tyrosine protein kinase ZAP70; Tyrosine-protein kinase ZAP-70; TZK; ZAP 70; ZAP70; ZAP70_HUMAN; Zeta chain associated protein kinase 70kD; Zeta chain associated protein kinase 70kDa; Zeta chain associated protein kinase 70kDa isoform 1; Zeta chain associated protein kinase 70kDa isoform 2; Zeta chain of T cell receptor associated protein kinase 70; Zeta chain TCR associated protein kinase 70kD; Zeta chain TCR associated protein kinase 70kDa

Target Background

This gene encodes an enzyme belonging to the protein tyrosine kinase family, and it plays a role in T-cell development and lymphocyte activation. This enzyme, which is phosphorylated on tyrosine residues upon T-cell antigen receptor (TCR) stimulation, functions in the initial step of TCR-mediated signal transduction in combination with the Src family kinases, Lck and Fyn. This enzyme is also essential for thymocyte development. Mutations in this gene cause selective T-cell defect, a severe combined immunodeficiency disease characterized by a selective absence of CD8-positive T-cells. Two transcript variants that encode different isoforms have been found for this gene.