TRA (T cell Receptor Alpha)

2000-07-01   Marie-Paule Lefranc  

IMGT, LIGM, IGH, UPR CNRS 1142, 141 rue de la Cardonille, 34396 Montpellier Cedex 5, France

Identity

HGNC
TRA
LOCATION
14q11.2
IMAGE
Atlas Image
LEGEND
for complete Figure, see: chromosome 14, IMGT (The International ImMunoGeneTics information system ®) © Copyright 1995-2017 IMGT, IMGT is a CNRS trademark
IMAGE
Atlas Image
LEGEND
TRA (T cell Receptor Alpha) Top: Probe bA60K12 - Courtesy Mariano Rocchi; Bottom: Hybridization with Vysis TRA/D Break Apart probe (Abbott Molecular, US) showing the gene on 14q11.2 (red-green or a fused yellow signal) - Courtesy Adriana Zamecnikova.
LOCUSID
6955
ALIAS
IMD7,TCRA,TRA@

DNA/RNA

Atlas Image
TRA/TRD
V-GENE: Green box: Functional; Red: Pseudogene.
D-GENE: Blue: Functional.
J-GENE: Yellow: Functional; Pale yellow: Open reading frame; Red: pseudogene.
C-GENE: Blue: Functional.
for complete Figure, see: locus TRA, IMGT (The International ImMunoGeneTics information system ®) © Copyright 1995-2017 IMGT, IMGT is a CNRS trademark

Description

  • The human TRA locus at 14q11.2 spans 1000 kilobases (kb). It consists of 54 TRAV genes belonging to 41 subgroups, 61 TRAJ segments localized on 71 kb, and a unique TRAC gene.
  • The most 5 TRAV genes occupy the most centromeric position, whereas the TRAC genes, 3 of the locus, is the most telomeric gene in the TRA locus.
  • The organization of the TRAJ segments on a large area is quite unusual and has not been observed in the other immunoglobulin or T cell receptor loci.
  • Moreover the TRD locus is nestled in the TRA locus between the TRAV and TRAJ segments. V-J-rearrangements in the TRA locus therefore result in deletion of the TRD genes localized on the same chromosome. That deletion occurs in two steps, that is a deletion of the TRD genes, involving specific sequences located upstream from TRDC (sequence pseudo J alpha) would take place before the TRAV-J rearrangement.
  • The potentiel genomic TRA repertoire comprises 45-47 functional TRAV genes belonging to 33-35 subgroups, 50 functional TRAJ segments, and the unique TRAC gene.
  • Among the variable genes are included five genes designated as TRAV/DV which belong to five different subgroups and which have been found rearranged either to TRAJ or to TRDD segments and can therefore be used in the synthesis of alpha or delta chains.
  • The total number of human TRA genes per haploid genome is 116 of which 96 to 98 genes are functional. Enhancer sequences have been characterized 4.5kb 3 from TRAC.
  • List of the human TRA genes

    • Mutations

    Note

    Mutations which correspond to allelic polymorphisms of the functional germline TRAV, TRAJ and TRAC genes are described in the IMGT database: (IMGT Repertoire>Alignments of alleles)

    Implicated in

    Entity name
    Translocations which frequently result from errors of the recombinase enzyme complex (RAG1, RAG2, etc.), which is responsable of the Immunoglobulin and T cell receptor V-J and V-D-J rearrangements. TRAV or TRAJ recombination signals or isolated heptamer are observed at the breakpoints.
    Atlas Image
    TRA (T cell Receptor Alpha) Hybridization with Vysis TRA/D Break Apart probe (Abbott Molecular, US) showing the gene on 14q11.2 (red-green or a fused yellow signal) - Courtesy Adriana Zamecnikova.
    Entity name
    t(1;14)(p32;q11); involve TRA - TAL1 in 1p32
    Prognosis
    median survival > 5 yrs in children
    Entity name
    t(8;14)(q24;q11); involve TRA - MYC in 8q24
    Disease
    T-cell acute lymphocytic leukemia (ALL); rare
    Entity name
    t(10;14)(q24;q11); involve TRA - HOX11 in 10q24
    Disease
    T- cell acute lymphoblastic leukemia (ALL) and non-Hodgkin lymphoma (NHL)
    Prognosis
    not unfavourable
    Entity name
    t(11;14)(p13;q11); involve RBTN2 in 11p13
    Disease
    T-cell acute lymphocytic leukemia (ALL)
    Entity name
    t(14;14)(q11;q32), inv(14)(q11q32); involveTRA - TCL1 in 14q32
    Disease
    T-cell prolymphocytic leukemia (T-PLL) and adult T cell leukemia/lymphoma
    Prognosis
    poor
    Entity name
    t(14;21)(q11;q22); involve TRA - OLIG2 in 21q22
    Disease
    T-cell acute lymphoblastic leukemia (ALL)
    Prognosis
    unknown

    Breakpoints

    Atlas Image

    Article Bibliography

    Pubmed IDLast YearTitleAuthors
    95194061998Crystal structure of p14TCL1, an oncogene product involved in T-cell prolymphocytic leukemia, reveals a novel beta-barrel topology.Hoh F et al

    Other Information

    Locus ID:

    NCBI: 6955
    HGNC: 12027

    Variants:

    dbSNP: 6955
    ClinVar: 6955
    COSMIC: TRA

    References

    Pubmed IDYearTitleCitations
    387231052024Type B thymomas in patients with myasthenia gravis display a distinctive pattern of αβ TCR and IL-7 receptor α expression on CD4(+)CD8(+) thymocytes.0
    387231052024Type B thymomas in patients with myasthenia gravis display a distinctive pattern of αβ TCR and IL-7 receptor α expression on CD4(+)CD8(+) thymocytes.0
    371537422023Selective ablation of TRA-1-60(+) pluripotent stem cells suppresses tumor growth of prostate cancer.0
    371537422023Selective ablation of TRA-1-60(+) pluripotent stem cells suppresses tumor growth of prostate cancer.0
    334822002021T cell receptor-dependent S-acylation of ZAP-70 controls activation of T cells.9
    336164772021Electrostatic Complementarity of T-Cell Receptor-Alpha CDR3 Domains and Mutant Amino Acids Is Associated with Better Survival Rates for Sarcomas.4
    334822002021T cell receptor-dependent S-acylation of ZAP-70 controls activation of T cells.9
    336164772021Electrostatic Complementarity of T-Cell Receptor-Alpha CDR3 Domains and Mutant Amino Acids Is Associated with Better Survival Rates for Sarcomas.4
    312377112020The 3A6-TCR/superagonist/HLA-DR2a complex shows similar interface and reduced flexibility compared to the complex with self-peptide.0
    315897932020Geometrical characterization of T cell receptor binding modes reveals class-specific binding to maximize access to antigen.12
    325328172020T cell receptor interactions with human leukocyte antigen govern indirect peptide selectivity for the cancer testis antigen MAGE-A4.14
    312377112020The 3A6-TCR/superagonist/HLA-DR2a complex shows similar interface and reduced flexibility compared to the complex with self-peptide.0
    315897932020Geometrical characterization of T cell receptor binding modes reveals class-specific binding to maximize access to antigen.12
    325328172020T cell receptor interactions with human leukocyte antigen govern indirect peptide selectivity for the cancer testis antigen MAGE-A4.14
    315584652019Mismatched related vs matched unrelated donors in TCRαβ/CD19-depleted HSCT for primary immunodeficiencies.21

    Citation

    Marie-Paule Lefranc

    TRA (T cell Receptor Alpha)

    Atlas Genet Cytogenet Oncol Haematol. 2000-07-01

    Online version: http://atlasgeneticsoncology.org/gene/39/tra