Download Leukaemia Section t(3;4)(q27;p13) Atlas of Genetics and Cytogenetics in Oncology and Haematology

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Transcript
Atlas of Genetics and Cytogenetics
in Oncology and Haematology
OPEN ACCESS JOURNAL AT INIST-CNRS
Leukaemia Section
Short Communication
t(3;4)(q27;p13)
Agnès Daudignon
Département d'Hematologie-Immunologie-Cytogenetique, Centre Hospitalier de Valenciennes, France
Published in Atlas Database: March 1998
Online updated version: http://AtlasGeneticsOncology.org/Anomalies/t34ID2055.html
DOI: 10.4267/2042/37439
This work is licensed under a Creative Commons Attribution-Non-commercial-No Derivative Works 2.0 France Licence.
© 1998 Atlas of Genetics and Cytogenetics in Oncology and Haematology
Protein
Expressed in hemopoietic tissues; GTPase of the Ras
superfamily; signal transduction.
Clinics and pathology
Disease
NHL
Phenotype / cell stem origin
Follicular centrocytic-centroblastic lymphoma.
Epidemiology
Only four cases available to date; all four patients were
female; median age: 57 yr.
Results of the chromosomal
anomaly
Hybrid gene
Description
5' RHOH - 3' BCL6 and 5' BCL6 - 3' RHOH, leading to
two fusion transcripts.
Cytogenetics
Fusion protein
Cytogenetics, morphological
Description
No fusion protein, but promoter exchange between both
partner genes.
In all 4 cases, t(14;18)(q32;q21) was present;
t(3;4)(q27;p13) may therefore be a secondary event.
Additional anomalies
References
Deletion of 6q was also found in all 4 cases, either as a
del(6q) or as a iso(6p); karyotypes were complex.
Dallery E, Galiègue-Zouitina S, Collyn-d'Hooghe M, Quief S,
Denis C, Hildebrand MP, Lantoine D, Deweindt C, Tilly H,
Bastard C, et al. TTF, a gene encoding a novel small G
protein, fuses to the lymphoma-associated LAZ3 gene by t(3;4)
chromosomal
translocation.
Oncogene
1995
Jun
1;10(11):2171-8.
Genes involved and Proteins
BCL6
Location: 3q27
DNA / RNA
10 exons.
Protein
Nuclear protein; contains 6 zinc fingers (C2H2 type);
transcription factor.
Daudignon A, Bisiau H, Le Baron F, Lai JL, Wetterwald M,
Galiègue-Zouitina S, Morel P, Duthilleul P. Four cases of
follicular lymphoma with t(14;18)(q32;q21) and t(3;4)(q27;p13)
with LAZ3 (BCL6) rearrangement. Cancer Genet Cytogenet
1999 Jun;111(2):157-60.
This article should be referenced as such:
Daudignon A. t(3;4)(q27;p13). Atlas Genet Cytogenet Oncol
Haematol.1998;2(3):94.
RHOH
Location: 4p13
Atlas Genet Cytogenet Oncol Haematol. 1998;2(3)
94