t(8;17)(q24;q22) ???BCL3/MYC

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Written2011-10Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

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1. Identity

Atlas_Id 1494
Note It is unlikely that the BCL3 gene (HGNC official name) is involved in this translocation with a breakpoint in 17q22, since BCL3 sits in 19q13.32 (coordonates: starts at 45251978 and ends at 45263301 bp from 19pter); the alternative would be a cryptic translocation, involving a cryptic inserted fragment of 19q13.32, including BCL3, within 17q22.

2. Clinics and Pathology

Disease Aggresive prolymphocytic leukemia
Epidemiology Only one case to date, with no clinical data.

3. Cytogenetics

Cytogenetics Morphological The karyotype also showed the classical t(14;18)(q32;q21), usually found in follicular lymphoma, a 12q+ and a Xp+, not otherwise described.

4. Genes involved and Proteins

Note As said above, it is unprobable that the MYC partner is BCL3.
Gene NameMYC v-myc myelocytomatosis viral oncogene homolog (avian)
Location 8q24.21
Protein MYC regulates the transcription of genes required to coordinate a range of cellular processes, including those essential for proliferation, growth, differentiation, apoptosis and self-renewal, and protein synthesis through ribosome biogenesis (van Riggelen et al., 2010).
Gene NameBCL3 (B-cell CLL/lymphoma 3)
Location 19q13.32
Protein BCL3 is mainly found in the nucleus. Protein which contains seven ankyrin repeats. Ankyrin repeats are found in IkB family members, including IkBa, IkBb, and IkBe. BCL3 is a member of the IkappaB family, whose proteins regulate the NFkappaB family of transcription factors. Component of a complex with a NF-kB p52-p52 homodimer Down-regulates inflammatory responses through limiting the transcription of NF-kB-dependent genes. Binds to NF-kB p50 and p52, Jab1, Pirin, Tip60 and Bard1. Bcl-3 is an adaptor protein (Dechend et al., 1999; Kreisel et al., 2011). Regulates genes involved in cell proliferation and apoptosis. NFkappaB plays a major role in B-cell development.

5. Result of the chromosomal anomaly

Hybrid gene
Description Disruption of MYC close to the first intron, with the decapitation of the first intron, replaced by a sequence of 1.7 kb, that the authors have called "BCL3".

6. 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.

7. Bibliography

The Bcl-3 oncoprotein acts as a bridging factor between NF-kappaB/Rel and nuclear co-regulators.
Dechend R, Hirano F, Lehmann K, Heissmeyer V, Ansieau S, Wulczyn FG, Scheidereit C, Leutz A.
Oncogene. 1999 Jun 3;18(22):3316-23.
PMID 10362352
Activation of MYC in a masked t(8;17) translocation results in an aggressive B-cell leukemia.
Gauwerky CE, Huebner K, Isobe M, Nowell PC, Croce CM.
Proc Natl Acad Sci U S A. 1989 Nov;86(22):8867-71.
PMID 2682663
Bcl3 prevents acute inflammatory lung injury in mice by restraining emergency granulopoiesis.
Kreisel D, Sugimoto S, Tietjens J, Zhu J, Yamamoto S, Krupnick AS, Carmody RJ, Gelman AE.
J Clin Invest. 2011 Jan 4;121(1):265-76. doi: 10.1172/JCI42596. Epub 2010 Dec 13.
PMID 21157041
MYC as a regulator of ribosome biogenesis and protein synthesis.
van Riggelen J, Yetil A, Felsher DW.
Nat Rev Cancer. 2010 Apr;10(4):301-9.
PMID 20332779

8. Citation

This paper should be referenced as such :
Huret, JL
t(8;17)(q24;q22) ???BCL3/MYC
Atlas Genet Cytogenet Oncol Haematol. 2012;16(3):236-237.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://atlasgeneticsoncology.usal.es/classic/Anomalies/t0817q24q22ID1494.html

9. Translocations implicated (Data extracted from papers in the Atlas)

 t(8;17)(q24;q22) ???BCL3/MYC

10. External links

Mitelman databaset(8;17)(q24;q22) [Case List]    t(8;17)(q24;q22) [Transloc-MCList] ???BCL3/MYC [Fusion-MCList]
arrayMap (UZH-SIB Zurich)[select an item]
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
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