Abstract:
:Homeobox containing transcription factors are frequently deregulated in human hematologic malignant diseases either indirectly through an abnormality of an upstream factor, or directly through rearrangement of the gene itself. Study of T-cell acute lymphoblastic leukemia identified the related non-clustered homeobox transcription factors, TLX1 and TLX3, as frequently ectopically expressed as a result of chromosomal translocations. We report the deregulation of a non-clustered homeobox gene in a new type of t(5;14)(q35;q11) translocation in a mature peripheral B-cell leukemia. This translocation results in the ectopic expression of the CSX1/NKX2-5 gene on chromosome 5q35 due to its juxtaposition to the TCR delta gene on chromosome 14q11. Expression of the CSX1/NKX2-5 protein conferred enhanced replating potential to transduced murine bone marrow cells. Our study establishes that deregulation of homeobox encoding genes is not restricted to acute leukemic proliferations, but is also observed in chronic malignant diseases.
journal_name
Haematologicajournal_title
Haematologicaauthors
Su X,Della-Valle V,Delabesse E,Azgui Z,Berger R,Merle-Béral H,Bernard OA,Nguyen-Khac Fdoi
10.3324/haematol.12595subject
Has Abstractpub_date
2008-07-01 00:00:00pages
1081-5issue
7eissn
0390-6078issn
1592-8721pii
haematol.12595journal_volume
93pub_type
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