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t(3;12)(q27;p12) LRMP/BCL6

Written2012-12Jean-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 2129

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics The t(3;12)(q27;p12) was found in a case of follicular lymphoma transformed to diffuse aggressive lymphoma, from a study with no individual data (Akasaka et al., 2003).

Genes involved and Proteins

Gene Name BCL6
Location 3q27.3
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). Role in germinal centers of lymphoid follicles. BCL6 prevents ATM and TP53 to induce apoptosis in response to DNA rearrangements such as somatic hypermutation and class switch recombination. Therefore essential for normal B cell development.
Gene Name LRMP
Location 12p12.1
Protein LRMP is an integral membrane protein of the endoplasmic reticulum (ER), oriented on the ER membrane facing the cytosol. It is a lymphoid-restricted gene that is expressed in a developmentally regulated fashion (Behrens et al., 1994). It may be involved in diabetes susceptibility (Grimm et al., 2003). A V141L polymorphism of LRMP has been found associated with a higher mortality in patients with lung cancer (Manenti et al., 2006). LRMP has recently been found involved in a translocation t(8;12)(q24;p12) with a LRMP/MYC fusion gene in non Hodgkin lymphoma (Singh et al., 2012).

Result of the chromosomal anomaly

Hybrid gene
Description Breakpoint in BCL6 first intron.
  

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

BCL6 gene translocation in follicular lymphoma: a harbinger of eventual transformation to diffuse aggressive lymphoma.
Akasaka T, Lossos IS, Levy R.
Blood. 2003 Aug 15;102(4):1443-8. Epub 2003 May 8.
PMID 12738680
 
Ambivalent role of BCL6 in cell survival and transformation.
Albagli-Curiel O.
Oncogene. 2003 Jan 30;22(4):507-16.
PMID 12555064
 
Jaw1, A lymphoid-restricted membrane protein localized to the endoplasmic reticulum.
Behrens TW, Jagadeesh J, Scherle P, Kearns G, Yewdell J, Staudt LM.
J Immunol. 1994 Jul 15;153(2):682-90.
PMID 8021504
 
Lrmp and Bcat1 are candidates for the type I diabetes susceptibility locus Idd6.
Grimm CH, Rogner UC, Avner P.
Autoimmunity. 2003 Jun;36(4):241-6.
PMID 14563018
 
A V141L polymorphism of the human LRMP gene is associated with survival of lung cancer patients.
Manenti G, Galbiati F, Pettinicchio A, Spinola M, Piconese S, Leoni VP, Conti B, Ravagnani F, Incarbone M, Pastorino U, Dragani TA.
Carcinogenesis. 2006 Jul;27(7):1386-90. Epub 2006 Jan 12.
PMID 16410263
 
Identification of a novel non-immunoglobulin/MYC translocation t(8;12)(q24;p12) involving the LRMP gene in a primary B-cell lymphoma. A case report.
Singh RR, Ben-Neriah S, Johnson NN, Connors JM, Gascoyne RD, Horsman DE.
Leuk Res. 2012 Jan;36(1):e22-4. doi: 10.1016/j.leukres.2011.09.020. Epub 2011 Oct 11.
PMID 21996557
 

Citation

This paper should be referenced as such :
Huret JL
t(3;12)(q27;p12) LRMP/BCL6;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0312q27p12ID2129.html


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

Genes BCL6

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;12)(q27;p12) LRMP/BCL6

External links

arrayMap (UZH-SIB Zurich)LRMP (12p12.1) BCL6 (3q27.3)

LRMP (12p12.1) BCL6 (3q27.3)

Mitelman databaset(3;12)(q27;p12) [Case List]    t(3;12)(q27;p12) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseLRMP/BCL6 [MCList]  LRMP (12p12.1) BCL6 (3q27.3)
Mitelman databaseLRMP/BCL6 [MCList]  LRMP (12p12.1) BCL6 (3q27.3)
 
Disease databaset(3;12)(q27;p12) LRMP/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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