Gene: [01q2/TCR2Z] T cell receptor, signal T3-zeta polypeptide (TiT3 complex); antigen CD3Z/T3, zeta polypeptide (T cell receptor); immunodeficiency combined (low expression T3-zeta chain); [CD3Z T3Z ]

COM

In the GDB and HGM Catalogue, T3 element genes are designated as CD3 (according to the common name for many membrane-associated glycoproteins of T and B cells that are markers for separate differentiation stages: luster ifferentiation antigen, N), whereas Ti element genes are designated as TCR (T ell eceptor). The GEM Catalogue unites all these genes under the common code TCR, adding '1' for the receptor Ti peptides (TCR1A,B,D,G) and '2' for the signal T3 ones (TCR2D,E,G,W,Z)."

FAG

Four different polypeptides that can form the T3 element have been identified: gamma (GEM:11q233/TCR2G), delta (GEM:11q233/TCR2D), epsilon (GEM:11q233/TCR2D), and zeta (this gene). Another protein, omega, is included in this group (mainly by convention), since it associates with the T3 element during its assembly in the endoplasmic reticulum. Three genes of this group form a cluster in chromosome 11q. The gene for zeta peptide is located in chromosome 1q. The omega peptide gene is not mapped yet (GEM:00.0/TCR2W)."

EXP

CD3H (eta) and CD3Q (theta) peptides are produced by alternative splicing of the same gene TCR2Z."

MOP

The T3 element is presumably a trimer and is noncovalently associated with a transmembrane domain of the Ti element."

REL

See FAM:TCR2/00.0.

REF

FOG,PAT "Alarcon &: New Engl J Med, 319, N18, 1203-1208, 1988
EVO,SEQ "Weissman &: Science, 239, (26 Feb), 1018-1021, 1988
FUN,MOP "Weissman &: Nature, 324, 480-482, 1986

KEY

imm, onc, recp, hem

CLA

coding, basic

LOC

01 q22-23

MIM

MIM: 186780

SYN

CD3Z T3Z

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