ZAP70

Tyrosine-protein kinase ZAP-70 ;
70 kDa zeta-chain associated protein ;
Syk-related tyrosine kinase ;

catalytic activity:ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate.,disease:Defects in ZAP70 are the cause of selective T-cell defect (STD) [MIM:176947]. STD is an autosomal recessive form of severe combined immunodeficiency characterized by a selective absence of CD8-type T-cells.,domain:The SH2 domain binds to the phosphorylated tyrosine-based activation motif (TAM) of CD3Z.,function:Plays a role in T-cell development and lymphocyte activation. Essential for TCR-mediated IL-2 production. Isoform 1 induces TCR-mediated signal transduction, isoform 2 does not.,online information:ZAP70 mutation db,PTM:Phosphorylated on tyrosine residues upon T-cell antigen receptor (TCR) stimulation. Tyr-319 phosphorylation is essential for full activity.,similarity:Belongs to the protein kinase superfamily. Tyr protein kinase family. SYK/ZAP-70 subfamily.,similarity:Contains 1 protein kinase domain.,similarity:Contains 2 SH2 domains.,subcellular location:After antigen stimulation, isoform 1 concentrates at the immunological synapse and isoform 2 remains cytoplasmic.,subunit:Interacts with SLA2 when it is phosphorylated. Interacts with CD3Z and with phosphorylated NFAM1. Interacts with CBLB (By similarity). Interacts with CBL and SLA when it is phosphorylated. The association with SLA (or SLA2) and CBL probably leads to its destruction. Interacts with SHB. Interacts with DEF6 (By similarity). Interacts with FCRL3.,tissue specificity:Expressed in T- and natural killer cells.,

cell activation, activation of immune response, immune response-activating cell surface receptor signaling pathway,immune system development, leukocyte differentiation, positive regulation of immune system process, regulation of leukocyte activation, positive regulation of leukocyte activation, immune response-activating signal transduction,immune response-regulating signal transduction, immune response-regulating cell surface receptor signaling pathway,protein amino acid phosphorylation, phosphorus metabolic process, phosphate metabolic process, immune response,cell surface receptor linked signal transduction, intracellular signaling cascade, protein kinase cascade, positive regulation of cell proliferation, positive regulation of signal transduction, positive regulation of cell communication,phosphorylation, peptidyl-tyrosine phosphorylation, peptidyl-tyrosine modification, hemopoiesis, lymphocyte differentiation, T cell differentiation, regulation of mononuclear cell proliferation, positive regulation of mononuclear cell proliferation, T cell differentiation in the thymus, positive regulation of T cell proliferation, T cell activation, regulation of cell proliferation, regulation of T cell proliferation, beta selection, positive T cell selection, negative T cell selection, T cell selection, positive thymic T cell selection, negative thymic T cell selection, thymic T cell selection, leukocyte activation, regulation of T cell differentiation, positive regulation of T cell differentiation, positive regulation of cell differentiation, regulation of lymphocyte differentiation, positive regulation of lymphocyte differentiation, alpha-beta T cell activation, alpha-beta T cell differentiation, regulation of alpha-beta T cell activation, positive regulation of alpha-beta T cell activation, regulation of alpha-beta T cell differentiation, positive regulation of alpha-beta T cell differentiation, regulation of alpha-beta T cell proliferation, positive regulation of alpha-beta T cell proliferation,lymphocyte activation, protein amino acid autophosphorylation, hemopoietic or lymphoid organ development, positive regulation of response to stimulus, regulation of lymphocyte proliferation, positive regulation of lymphocyte proliferation, positive regulation of immune response, regulation of calcium-mediated signaling, positive regulation of calcium-mediated signaling, antigen receptor-mediated signaling pathway, T cell receptor signaling pathway,regulation of T cell activation, regulation of cell activation, positive regulation of cell activation, positive regulation of T cell activation, positive regulation of developmental process, regulation of lymphocyte activation, positive regulation of lymphocyte activation, regulation of leukocyte proliferation, positive regulation of leukocyte proliferation,

Cytoplasm . Cell membrane ; Peripheral membrane protein . In quiescent T-lymphocytes, it is cytoplasmic. Upon TCR activation, it is recruited at the plasma membrane by interacting with CD247/CD3Z. Colocalizes together with RHOH in the immunological synapse. RHOH is required for its proper localization to the cell membrane and cytoskeleton fractions in the thymocytes (By similarity). .

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