The ITAM domains mediate interaction with SHB.
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).
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.
Phosphorylated on Tyr residues after T-cell receptor triggering by LCK in association with CD4/CD8.
Belongs to the CD3Z/FCER1G family.
CD3Z is expressed in normal lymphoid tissue and in peripheral blood mononuclear cells (PBMCs) (PubMed:11722641).
CD247, CD3Z, T3Z, TCRZ, T-cell surface glycoprotein CD3 zeta chain, T-cell receptor T3 zeta chain
Proteins
Immunology & Infectious Disease
18696Da
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