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t(3;3)(q27;q27) ST6GAL1/BCL6

del(3)(q27q27) ST6GAL1/BCL6

Written2012-06Jean-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 2128

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics Two cases of follicular lymphoma transformed to diffuse aggressive lymphoma, from a study with no individual data (Akasaka et al., 2003; cited by Ohno, 2006).

Cytogenetics

Cytogenetics Morphological Cryptic rearrangement.

Genes involved and Proteins

Gene Name ST6GAL1
Location 3q27.3
Note ST6GAL1 (ST6 beta-galactosamide alpha-2,6-sialyltranferase 1) is also known under the name of SIAT1, and was described as such in the Alaska's report.
Protein 406 amino acids; glycosyltransferase (Wu et al., 2011).
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.

Result of the chromosomal anomaly

Hybrid gene
Note ST6GAL1 and BCL6 are normally separated by 699.5 kb.
  

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
 
Pathogenetic and clinical implications of non-immunoglobulin ; BCL6 translocations in B-cell non-Hodgkin's lymphoma.
Ohno H.
J Clin Exp Hematop. 2006 Nov;46(2):43-53. (REVIEW)
PMID 17142954
 
Universal phosphatase-coupled glycosyltransferase assay.
Wu ZL, Ethen CM, Prather B, Machacek M, Jiang W.
Glycobiology. 2011 Jun;21(6):727-33. Epub 2010 Nov 15.
PMID 21081508
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;3)(q27;q27) ST6GAL1/BCL6 - del(3)(q27q27) ST6GAL1/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2012;16(12):925-926.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0303q27q27ST6GAL1BCL6ID2128.html


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

Genes BCL6

Translocations implicated (Data extracted from papers in the Atlas)

 del(3)(q27q27)
 t(3;3)(q27;q27) ST6GAL1/BCL6

External links

arrayMap (UZH-SIB Zurich)ST6GAL1 (3q27.3) BCL6 (3q27.3)

Mitelman databasedel(3)(q27q27) [Case List]    del(3)(q27q27) [Association List] Mitelman database (CGAP - NCBI)
Mitelman databaset(3;3)(q27;q27) [Case List]    t(3;3)(q27;q27) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseST6GAL1/BCL6 [MCList]  ST6GAL1 (3q27.3) BCL6 (3q27.3)
 
Disease databaset(3;3)(q27;q27) ST6GAL1/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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