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t(1;7)(p32;q34) TRB/TAL1

t(1;14)(p32;q11) TRA/TAL1

1p32 rearrangements

Written1998-01Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9837/3 T lymphoblastic leukaemia/lymphoma
Atlas_Id 1047
Note the two chromosome anomalies are variants of each other, and they share identical features
 
  t(1;14)(p32;q11) G-banding (top two) and fluorescence in situ hybridization with LSI TRA/TRD break-apart probe (Vysis, Abott Molecular, US) revealing juxtaposition of telomeric gene sequences (green) from chromosome 14 to der(1) chromosome as a result of the translocation - Courtesy Adriana Zamecnikova; t(1;14)(p32;q11) G-banding (middle) - Courtesy Diane H. Norback, Eric B. Johnson, and Sara Morrison-Delap, UW Cytogenetic Services; and t(1;14)(p32;q11) R-banding (bottom) - Courtesy Isabelle Radford-Weiss.

Clinics and Pathology

Disease T- cell ALL
Epidemiology rare findings; t(1;14) is found in approximately 3% of T-ALL; t(1;7) is rarer (status 3: < 5 cases); however, TAL1 rearrangements, all together (being mostly submicroscopic deletions without visible 1p32 involvement), occurs in 15-25% of T-ALL; male predominance (as is classical in T-cell ALL)
Clinics organomegaly; high WBC (median 200X 109/l)

Cytogenetics

Additional anomalies t(1;14) is found solely in about half cases, and accompanied by del(6q) in nearly half cases as well

Genes involved and Proteins

Gene NameTAL1 (T-cell acute leukemia 1)
Location 1p33
Dna / Rna complex alternate splicing
Protein contains a basic Helix-Loop-Helix (DNA binding) domain; forms heterodimers; transcription factor; role in haematopoietic cell differentiation
Gene NameTRA (T cell Receptor Alpha)
Location 14q11.2
Gene NameTRB (T cell Receptor Beta)
Location 7q34

Result of the chromosomal anomaly

Fusion Protein
 
Description in most cases (breakpoints between exons 2B and 3 of TAL1), 3' TAL1 joins variable and diversity segments of TCR on der(14); in the few cases where the breakpoint is within exon 6 of TAL1 or 3' from it, constant segments of TCR join TAL1 on der(1)
  

Bibliography

Clinical features and outcome of T-cell acute lymphoblastic leukemia in childhood with respect to alterations at the TAL1 locus: a Pediatric Oncology Group study.
Bash RO, Crist WM, Shuster JJ, Link MP, Amylon M, Pullen J, Carroll AJ, Buchanan GR, Smith RG, Baer R
Blood. 1993 ; 81 (8) : 2110-2117.
PMID 8471769
 

Citation

This paper should be referenced as such :
Huret, JL
t(1;7)(p32;q34)
Atlas Genet Cytogenet Oncol Haematol. 1998;2(1):20-21.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0114ID1047.html


Other genes implicated (Data extracted from papers in the Atlas) [ 4 ]

Genes TAL1 TRA TRB TRD

Translocations implicated (Data extracted from papers in the Atlas)

 t(1;14)(p32;q11) TRA/TAL1
 t(1;7)(p32;q34) TRB/TAL1

External links

Mitelman databaset(1;14)(p32;q11) [Case List]    t(1;14)(p32;q11) [Association List] Mitelman database (CGAP - NCBI)
Mitelman databaset(1;7)(p32;q34) [Case List]    t(1;7)(p32;q34) [Association List] Mitelman database (CGAP - NCBI)
arrayMapTopo ( C42) Morph ( 9837/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaset(1;7)(p32;q34) TRB/TAL1
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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indexed on : Mon Sep 18 17:19:06 CEST 2017


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