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. ,
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