CD247 phospho Y83
Domain
The ITAM domains mediate interaction with SHB.
Function
Part of the TCR-CD3 complex present on T-lymphocyte cell surface that plays an essential role in adaptive immune response. When antigen presenting cells (APCs) activate T-cell receptor (TCR), TCR-mediated signals are transmitted across the cell membrane by the CD3 chains CD3D, CD3E, CD3G and CD3Z. All CD3 chains contain immunoreceptor tyrosine-based activation motifs (ITAMs) in their cytoplasmic domain. Upon TCR engagement, these motifs become phosphorylated by Src family protein tyrosine kinases LCK and FYN, resulting in the activation of downstream signaling pathways (PubMed:1384049, PubMed:1385158, PubMed:2470098, PubMed:7509083). CD3Z ITAMs phosphorylation creates multiple docking sites for the protein kinase ZAP70 leading to ZAP70 phosphorylation and its conversion into a catalytically active enzyme (PubMed:7509083). Plays an important role in intrathymic T-cell differentiation. Additionally, participates in the activity-dependent synapse formation of retinal ganglion cells (RGCs) in both the retina and dorsal lateral geniculate nucleus (dLGN) (By similarity).
Involvement in disease
Immunodeficiency 25
IMD25
An immunological deficiency characterized by T-cells impaired immune response to alloantigens, tetanus toxoid and mitogens.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
Phosphorylated on Tyr residues after T-cell receptor triggering by LCK in association with CD4/CD8.
Sequence Similarities
Belongs to the CD3Z/FCER1G family.
Tissue Specificity
CD3Z is expressed in normal lymphoid tissue and in peripheral blood mononuclear cells (PBMCs) (PubMed:11722641).
Cellular localization
- Cell membrane
- Single-pass type I membrane protein
Alternative names
CD247, CD3Z, T3Z, TCRZ, T-cell surface glycoprotein CD3 zeta chain, T-cell receptor T3 zeta chain