CD3Zeta deficiency

Gene Information


HUGO name: CD247


  • CD3Z
  • T3Z
  • TCRZ


Reference sequences

Chromosomal location


Protein Information


Protein function:

Probable role in assembly and expression of the TCR complex as well as signal transduction upon antigen triggering.


The TCR/CD3 complex of T-lymphocytes consists of either a TCR alpha/beta or TCR gamma/delta heterodimer coexpressed at the cell surface with the invariant subunits of CD3 labeled gamma, delta, epsilon, zeta, and eta. Cd3-zeta forms either homodimers or heterodimers with CD3-eta. Interacts with Sla and Sla2. Interacts with Dock2 and Trat1. Interacts with HIV-1 Nef protein. Interacts with SHB.

Subcellular location:

Membrane; single-pass type i membrane protein.

Post-translational modification:

Phosphorylated on Tyr residues after T-cell receptor triggering (by similarity).

Alternative products:

Event=alternative splicing; named isoforms=2; name=cd-3-zeta; isoid=p20963-1; sequence=displayed; name=cd-3-eta; isoid=p20963-2; sequence=not described;


Belongs to the CD3Z/fcer1g family.

Structures (PDB)

1TCE .


ITAM 1 domain: 61-89

    ITAM 2 domain: 100-128

      ITAM 3 domain: 131-159

        Other features

        Signal peptide: 1-21

          T-cell surface glycoprotein cd3 zeta chain: 22-164

            Disulfide bond interchain: 32

              Other related resourses:


              Expression pattern for human

              Tissue Expression (%) Clones
              thymus 56.12 43:68776
              spleen 11.38 6:47318
              blood 4.38 3:61437
              uncharacterized_tissue 4.16 10:215582
              mixed 3.11 10:288723
              heart 2.61 2:68859
              muscle 2.50 3:107777
              prostate 2.25 3:119494
              lymph_node 2.16 2:83039
              embryonic_tissue 1.97 1:45612

              Animal models