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t(11;14)(p13;q11) TRD/LMO2

t(7;11)(q35;p13) TRB/LMO2

Written1998-09Chrystèle Bilhou-Nabera
Cytogénétique,Laboratoire d'Hématologie-Pr RAPHAEL, Pav BROCA - 4me étage, 78 rue du Général Leclerc, 94275 LE KREMLIN-BICETRE, 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 1070
Note this t(11;14) must be not confused with the t(11;14)(p15;q11) associated with an immature immunophenotype (CD3-, CD4-, CD8-) and involving respectively RTBN1 gene and TRD locus.
 
  t(11;14)(p13;q11) left: G- banding; top - Courtesy Diane H. Norback, Eric B. Johnson, Sara Morrison-Delap UW Cytogenetic Services; middle - Courtesy Jean-Luc Lai and Alain Vanderhaegen; center left: R- banding; top - Courtesy Jean-Luc Lai and Alain Vanderhaegen; middle and bottom: - Courtesy Lucienne Michaux, Peter Meeus and Peter Vandenberghe; right: FISH - Courtesy Lucienne Michaux, Peter Meeus and Peter Vandenberghe.

Clinics and Pathology

Disease T-ALL
Epidemiology 5-10% of childhood T-ALL

Cytogenetics

Additional anomalies +17; to be noted that a Ph chromosome (m-BCR) has been found in one case of T-ALL
Variants t(11;14)(p13;q11) and t(7;11)(q35;p13) are variant translocations of each other

Genes involved and Proteins

Gene Name LMO2
Location 11p13
Protein cystein-rich protein with two tandemly arranged zinc binding LIM-domain motifs: named Lom2; Lmo2 directly interacts with the basic-loop-helix protein Tal1/Scl and the GATA DNA protein Gata-1; central role in adult hematopoietic pathway regulation.
Gene Name TRA/D
Location 14q11
Gene Name TRB
Location 7q35

Result of the chromosomal anomaly

Hybrid gene
Description chromosomal breakpoints occur 25 Kb upstream RBTN2 gene, in a presumed transcriptional start site, inducing truncation of the promoter/control region and leading to inappropriate Lmo2 level especially in T-cells (abnormal T-cell differentiation).
  
Fusion Protein
Oncogenesis Lmo2 is activated after chromosomal translocation by association with either the TRA/D or the TRB.
  

To be noted

CELL LINE with t(11;14)(p13;q11) : KOPT-K1; the breakpoints occur : - on chromosome 11 in an Alu-rich region, between two Alu sequences, 160 Kb-closed from RTBN2; - on chromosome 14 within Jd1; RTBN2 is highly expressed in KOPT-K1.

Bibliography

The rhombotin family of cysteine-rich LIM-domain oncogenes: distinct members are involved in T-cell translocations to human chromosomes 11p15 and 11p13.
Boehm T, Foroni L, Kaneko Y, Perutz MF, Rabbitts TH
Proceedings of the National Academy of Sciences of the United States of America. 1991 ; 88 (10) : 4367-4371.
PMID 2034676
 
Expression of rhombotin 2 in normal and leukaemic haemopoietic cells.
Dong WF, Billia F, Atkins HL, Iscove NN, Minden MD
British journal of haematology. 1996 ; 93 (2) : 280-286.
PMID 8639417
 
T-cell acute childhood lymphoblastic leukemia with chromosome 14 q 11 anomaly: a morphologic, immunologic, and cytogenetic analysis of 10 patients.
Lampert F, Harbott J, Ritterbach J, Ludwig WD, Fonatsch C, Schwamborn D, Stier B, Gnekow A, Gerein V, Stollmann B
Blut. 1988 ; 56 (3) : 117-123.
PMID 3258538
 
Cytogenetics of childhood T-cell leukemia.
Raimondi SC, Behm FG, Roberson PK, Pui CH, Rivera GK, Murphy SB, Williams DL
Blood. 1988 ; 72 (5) : 1560-1566.
PMID 3263151
 
The LIM-only protein Lmo2 is a bridging molecule assembling an erythroid, DNA-binding complex which includes the TAL1, E47, GATA-1 and Ldb1/NLI proteins.
Wadman IA, Osada H, Grütz GG, Agulnick AD, Westphal H, Forster A, Rabbitts TH
The EMBO journal. 1997 ; 16 (11) : 3145-3157.
PMID 9214632
 
New chromosomal translocations correlate with specific immunophenotypes of childhood acute lymphoblastic leukemia.
Williams DL, Look AT, Melvin SL, Roberson PK, Dahl G, Flake T, Stass S
Cell. 1984 ; 36 (1) : 101-109.
PMID 6607116
 

Citation

This paper should be referenced as such :
Bilhou-Nabera, C
t(11;14)(p13;q11) - t(7;11)(q35;p13)
Atlas Genet Cytogenet Oncol Haematol. 1999;3(1):22-23.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t1114ID1070.html


Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes LMO2

Translocations implicated (Data extracted from papers in the Atlas)

 t(11;14)(p13;q11) TRD/LMO2
 t(7;11)(q35;p13) TRB/LMO2

External links

Mitelman databaset(11;14)(p13;q11) [Case List]    t(11;14)(p13;q11) [Association List] Mitelman database (CGAP - NCBI)
Mitelman databaset(7;11)(q35;p13) [Case List]    t(7;11)(q35;p13) [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(11;14)(p13;q11) TRD/LMO2
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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