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t(3;6)(q27;q14) SNHG5/BCL6

Written2012-11Jean-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 2133

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics The t(3;6)(q27;q14) was found in a human diffuse large B-cell lymphoma cell line (Tanaka et al., 2000), and 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 SNHG5
Location 6q14.3
Protein SNHG5 is also known as U50HG. SNHG5 exons do not encode a polypeptide product (small nucleolar RNA (snoRNA) sequence). SNHG5 is composed of six exons. It possesses an oligopyrimidine tract that is characteristic of the 5'-terminal oligopyrimidine (5'TOP gene family) which have been shown to regulate cell growth.

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
 
Intronic U50 small-nucleolar-RNA (snoRNA) host gene of no protein-coding potential is mapped at the chromosome breakpoint t(3;6)(q27;q15) of human B-cell lymphoma.
Tanaka R, Satoh H, Moriyama M, Satoh K, Morishita Y, Yoshida S, Watanabe T, Nakamura Y, Mori S.
Genes Cells. 2000 Apr;5(4):277-87.
PMID 10792466
 

Citation

This paper should be referenced as such :
Huret JL
t(3;6)(q27;q14) SNHG5/BCL6;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0306q27q14ID2133.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;6)(q27;q14) SNHG5/BCL6

External links

arrayMap (UZH-SIB Zurich)SNHG5 (6q14.3) BCL6 (3q27.3)

SNHG5 (6q14.3) BCL6 (3q27.3)

Mitelman databaset(3;6)(q27;q14) [Case List]    t(3;6)(q27;q14) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseSNHG5/BCL6 [MCList]  SNHG5 (6q14.3) BCL6 (3q27.3)
Mitelman databaseSNHG5/BCL6 [MCList]  SNHG5 (6q14.3) BCL6 (3q27.3)
 
Disease databaset(3;6)(q27;q14) SNHG5/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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