Download Leukaemia Section t(8;17)(q24;q22) ???BCL3/MYC Atlas of Genetics and Cytogenetics

Atlas of Genetics and Cytogenetics
in Oncology and Haematology
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Leukaemia Section
Short Communication
t(8;17)(q24;q22) ???BCL3/MYC
Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France
(JLH)
Published in Atlas Database: October 2011
Online updated version : http://AtlasGeneticsOncology.org/Anomalies/t0817q24q22ID1494.html
DOI: 10.4267/2042/47288
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence.
© 2012 Atlas of Genetics and Cytogenetics in Oncology and Haematology
apoptosis and self-renewal, and protein synthesis
through ribosome biogenesis (van Riggelen et al.,
2010).
Identity
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.
BCL3
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 NFkB 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.
Clinics and pathology
Disease
Aggresive prolymphocytic leukemia
Epidemiology
Only one case to date, with no clinical data.
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.
Result of the chromosomal
anomaly
Genes involved and proteins
Hybrid gene
Note
As said above, it is unprobable that the MYC partner is
BCL3.
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".
MYC
References
Protein
MYC regulates the transcription of genes required to
coordinate a range of cellular processes, including
those essential for proliferation, growth, differentiation,
Atlas Genet Cytogenet Oncol Haematol. 2012; 16(3)
Gauwerky CE, Huebner K, Isobe M, Nowell PC, Croce CM.
Activation of MYC in a masked t(8;17) translocation results in
an aggressive B-cell leukemia. Proc Natl Acad Sci U S A. 1989
Nov;86(22):8867-71
236
t(8;17)(q24;q22) ???BCL3/MYC
Huret JL
Dechend R, Hirano F, Lehmann K, Heissmeyer V, Ansieau S,
Wulczyn FG, Scheidereit C, Leutz A. The Bcl-3 oncoprotein
acts as a bridging factor between NF-kappaB/Rel and nuclear
co-regulators. Oncogene. 1999 Jun 3;18(22):3316-23
Kreisel D, Sugimoto S, Tietjens J, Zhu J, Yamamoto S,
Krupnick AS, Carmody RJ, Gelman AE. Bcl3 prevents acute
inflammatory lung injury in mice by restraining emergency
granulopoiesis. J Clin Invest. 2011 Jan 4;121(1):265-76
van Riggelen J, Yetil A, Felsher DW. MYC as a regulator of
ribosome biogenesis and protein synthesis. Nat Rev Cancer.
2010 Apr;10(4):301-9
This article should be referenced as such:
Atlas Genet Cytogenet Oncol Haematol. 2012; 16(3)
Huret JL. t(8;17)(q24;q22) ???BCL3/MYC. Atlas
Cytogenet Oncol Haematol. 2012; 16(3):236-237.
237
Genet