del(11)(p12p13) LMO2

2007-07-01   Jules PP Meijerink  , Pieter Van Vlierberghe  

1.ErasmusMC/Sophia Children Hospital, Pediatric Oncology/Hematology, Rotterdam, The Netherlands

Clinics and Pathology

Disease

T-cell acute lymphoblastic leukemia (T-ALL).

Epidemiology

About 5% of T-ALL patients.

Prognosis

Currenlty, no relation between the cryptic deletion, del(11)(p12p13), and prognosis could be established. This could be due to the limited patient numbers in the study.

Note

The cryptic deletion, del(11)(p12p13) was identified using microarray-based comparative genome hybridisation (array-CGH). The deleted region is about 3 Mb in size and the telomeric breakpoint of these deletions is situated in or near the LMO2 oncogene. Variances in the centromeric breakpoints is detected.

Genes Involved and Proteins

Gene name
LMO2 (LIM domain only 2 (rhombotin-like 1))
Location
11p13
Protein description
LMO2 encodes a protein that participates in the transcription factor complex, which includes E2A, TAL1, GATA1, and LDB1 in erythroid cells. Within this transcription complex, LMO2 mediates the protein-protein interactions by recruiting LDB1, whereas TAL1, GATA1, and E2A regulate the binding to specific DNA target sites. This complex regulates the expression of several genes in various cellular backgrounds including C-KIT, EKLF, and RALDH. In normal T-cell development, LMO2 is expressed in immature CD4/CD8 double-negative thymocytes, and is down-regulated during T-cell maturation.

Article Bibliography

Pubmed IDLast YearTitleAuthors
168736702006The cryptic chromosomal deletion del(11)(p12p13) as a new activation mechanism of LMO2 in pediatric T-cell acute lymphoblastic leukemia.Van Vlierberghe P et al

Citation

Jules PP Meijerink ; Pieter Van Vlierberghe

del(11)(p12p13) LMO2

Atlas Genet Cytogenet Oncol Haematol. 2007-07-01

Online version: http://atlasgeneticsoncology.org/haematological/1351/del(11)(p12p13)