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t(3;6)(q27;p21) PIM1/BCL6

Written2009-08Jean-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
Atlas_Id 2088
Note t(3;6)(q27;p21) has been described in a few cases were PIM1/BCL6 rearrangement has not been ascertained, or were another hybrid gene has been uncovered.

Clinics and Pathology

Disease Non Hodgkin lymphoma (NHL).
Epidemiology Only one case available: a case of diffuse large B-cell lymphoma (DLBCL) (Yoshida et al., 1999).

Genes involved and Proteins

Gene Name BCL6
Location 3q27
Protein 706 amino acids; composed of a NH2-term BTB/POZ domain (amino acids 1-130 (32-99 according to Swiss-Prot)) which mediates homodimerization and protein-protein interactions with other corepressors (including HDAC1 and NCOR2/SMRT) to constitute a large repressing complex, another transcription repression domain (191-386), PEST sequences (300-417) with a KKYK motif (375-379), and six zinc finger at the C-term (518-541, 546-568, 574-596, 602-624, 630-652, 658-681), responsible for sequence specific DNA binding. Transcription repressor; recognizes the consensus sequence: TTCCT(A/C)GAA (Albagli-Curiel, 2003).
Gene Name PIM1
Location 6p21.2
Protein 404 amino acids; serine/threonine-protein kinase; regulated by hematopoietic cytokine receptors; synergy with c-MYC in cell proliferation and in apoptosis induction through an enhancement of the activation of caspase-3-like proteases; Cdc25A (cell cycle phosphatase) is a substrate for Pim-1

Result of the chromosomal anomaly

Hybrid gene
Description 5' PIM1 - 3' BCL6, but also 5' BCL6 - 3' PIM1; breakpoint in BCL6 between exon 1 and 2.
  

To be noted

Additional cases are needed to delineate the epidemiology of this rare entity:
you are welcome to submit a paper to our new Case Report section.

Bibliography

Ambivalent role of BCL6 in cell survival and transformation.
Albagli-Curiel O.
Oncogene. 2003 Jan 30;22(4):507-16.
PMID 12555064
 
Identification of heterologous translocation partner genes fused to the BCL6 gene in diffuse large B-cell lymphomas: 5'-RACE and LA - PCR analyses of biopsy samples.
Yoshida S, Kaneita Y, Aoki Y, Seto M, Mori S, Moriyama M.
Oncogene. 1999 Dec 23;18(56):7994-9.
PMID 10637510
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;6)(q27;p21) PIM1/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2010;14(7):694-694.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0306q27p21ID2088.html


Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes BCL6 PIM1

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;6)(q27;p21) PIM1/BCL6

External links

arrayMap (UZH-SIB Zurich)PIM1 (6p21.2) BCL6 (3q27.3)

PIM1 (6p21.2) BCL6 (3q27.3)

Mitelman databaset(3;6)(q27;p21) [Case List]    t(3;6)(q27;p21) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databasePIM1/BCL6 [MCList]  PIM1 (6p21.2) BCL6 (3q27.3)
TICdbPIM1/BCL6  PIM1 (6p21.2) BCL6 (3q27.3)
 
Disease databaset(3;6)(q27;p21) PIM1/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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indexed on : Fri Jan 27 12:50:34 CET 2017


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