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t(3;19)(q27;q13) NAPA/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 2130

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics Two cases available : a case of follicular lymphoma transformed to diffuse aggressive lymphoma, from a study with no individual data (Akasaka et al., 2003), and a male patient aged 60 years with a diffuse large B-cell lymphoma (Yunis et al., 1984).

Cytogenetics

Additional anomalies The case described by Yunis et al., 1984 showed a complex karyotype.

Genes involved and Proteins

Note Only the more recent case (Akasaka et al., 2003) was studied for gene rearrangements.
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 NAPA
Location 19q13.33
Protein 295 amino acids; SNARE protein found in the endoplasmic reticulum and implicated in protein trafficking, which possesses anti-apoptotic properties by promoting resistance to cisplatin in cancer cells by inducing the degradation of TP53 (Wu et al., 2011).

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
 
Silencing of the SNARE protein NAPA sensitizes cancer cells to cisplatin by inducing ERK1/2 signaling, synoviolin ubiquitination and p53 accumulation.
Wu ZZ, Sun NK, Chien KY, Chao CC.
Biochem Pharmacol. 2011 Dec 1;82(11):1630-40. Epub 2011 Aug 31.
PMID 21903092
 
Recurrent chromosomal defects are found in most patients with non-Hodgkin's-lymphoma.
Yunis JJ, Oken MM, Theologides A, Howe RB, Kaplan ME.
Cancer Genet Cytogenet. 1984 Sep;13(1):17-28.
PMID 6467179
 

Citation

This paper should be referenced as such :
Huret JL
t(3;19)(q27;q13) NAPA/BCL6;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0319q27q13NAPABCL6ID2130.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;19)(q27;q13) NAPA/BCL6

External links

arrayMap (UZH-SIB Zurich)NAPA (19q13.32) BCL6 (3q27.3)

NAPA (19q13.32) BCL6 (3q27.3)

Mitelman databaset(3;19)(q27;q13) [Case List]    t(3;19)(q27;q13) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseNAPA/BCL6 [MCList]  NAPA (19q13.32) BCL6 (3q27.3)
Mitelman databaseNAPA/BCL6 [MCList]  NAPA (19q13.32) BCL6 (3q27.3)
 
Disease databaset(3;19)(q27;q13) NAPA/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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indexed on : Fri Jun 30 11:23:06 CEST 2017


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