Atlas of Genetics and Cytogenetics in Oncology and Haematology


Home   Genes   Leukemias   Solid Tumours   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

X Y 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 NA
Taking over the Atlas
Dear Colleagues,
The Atlas, once more, is in great danger, and I will have to proceed to a collective economic lay-off of all the team involved in the Atlas before the begining of April 2015 (a foundation having suddenly withdrawn its commitment to support the Atlas). I ask you herein if any Scientific Society (a Society of Cytogenetics, of Clinical Genetics, of Hematology, or a Cancer Society, or any other...), any University and/or Hospital, any Charity, or any database would be interested in taking over the Atlas, in whole or in part. If taking charge of the whole lot is too big, a consortium of various actors could be the solution (I am myself trying to find partners). Could you please spread the information, contact the relevant authorities, and find partners.
Survival of the Atlas will be critically dependant upon your ability to find solutions (and urgently!).
Kind regards.
Jean-Loup Huret jlhuret@AtlasGeneticsOncology.org
Donations are also welcome
If each casual visitor gives 3 Euros or Dollars, the Atlas is saved in a week !
If each professional gives 100 Euros or Dollars once a year (now), the Atlas is saved in 2 weeks !
Don't let the Atlas imminent demise
Note: we send fiscal receipts for donations equal or above 50 Euros or Dollars

Donate (in Euros)

Donate (in US Dollars)


t(5;14)(q35;q32) BCL11B/TLX3

Identity

 
  t(5;14)(q35;q32) FISH - Courtesy Melanie Zenger and Claudia Haferlach.

Clinics and Pathology

Disease T cell acute lymphoblastic leukemia (ALL)
Phenotype / cell stem origin cortical T cell leukemia (CD1a+,CD10+)
Epidemiology frequent in T-cell ALL in children (in about 20% of childhood T-cell ALLs); less frequent in adult T-ALL. Not seen in B-cell ALL
Cytology FAB nomenclature: L1 or L2 ALL
Prognosis present data suggest that t(5;14)(q35;q32) is associated with poor outcome, but confirmatory data is necessary prior to conclude

Cytogenetics

Cytogenetics Morphological Cryptic translocation (banded karyotype). Often apparently normal karyotype with banding techniques.
Cytogenetics Molecular t(5;14)(q35;q32) can be detected with FISH techniques. Several probes may be used: chromosome painting, combination of painting probes and YAC, multicolor-FISH with adequate probes. The localization of the chromosomal breakpoint with BACs/PACs will be performed in a second step.
Additional anomalies variable

Genes involved and Proteins

Note The consequence of the translocation is the ectopic expression of the HOX11L2, gene normally located to 5q35, and normally not expressed in ALL without 5q rearrangement. The "deregulation" of HOX11L2 expression is thought to result from abnormal control of the gene by CTPI2, located to 14q32, as a consequence of the chromosomal rearrangement. The chromosome 5 breakpoint is usually located within the locus of another gene, RanBP17, often disrupted by the chromosomal rearrangement. The breakpoint on chromosome 5 is consequently distant from the gene abnormally expressed (HOX11L2).
Gene Name HOX11L2
Location 5q35
Protein homeobox domain; belongs to HOX 11 family

Result of the chromosomal anomaly

Fusion Protein
Description no fusion protein, but abnormal expression of HOX11L2
Oncogenesis HOX11L2 is transcriptionally activated, due to control by CITP2 regulatory sequences.
  

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

Genes BCL11B TLX3 VRK1

Translocations implicated (Data extracted from papers in the Atlas)

 t(5;14)(q35;q32) BCL11B/TLX3

External links

Mitelman database
t(5;14)(q35;q32) - Mitelman database (CGAP - NCBI)

Bibliography

A new recurrent and specific cryptic translocation, t(5;14)(q35;q32), is associated with expression of the Hox11L2 gene in T acute lymphoblastic leukemia.
Bernard OA, Busson-LeConiat M, Ballerini P, Mauchauffˆ© M, Della Valle V, Monni R, Nguyen Khac F, Mercher T, Penard-Lacronique V, Pasturaud P, Gressin L, Heilig R, Daniel MT, Lessard M, Berger R
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 2001 ; 15 (10) : 1495-1504.
PMID 11587205
 
Translocation t(5;14)(q35;q32) in three cases of childhood T cell acute lymphoblastic leukemia: a new recurring and cryptic abnormality.
Hˆ©lias C, Leymarie V, Entz-Werle N, Falkenrodt A, Eyer D, Costa JA, Cherif D, Lutz P, Lessard M
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 2002 ; 16 (1) : 7-12.
PMID 11840257
 
Gene expression signatures define novel oncogenic pathways in T cell acute lymphoblastic leukemia.
Ferrando AA, Neuberg DS, Staunton J, Loh ML, Huard C, Raimondi SC, Behm FG, Pui CH, Downing JR, Gilliland DG, Lander ES, Golub TR, Look AT
Cancer cell. 2002 ; 1 (1) : 75-87.
PMID 12086890
 
HOX11L2 expression defines a clinical subtype of pediatric T-ALL associated with poor prognosis.
Ballerini P, Blaise A, Busson-Le Coniat M, Su XY, Zucman-Rossi J, Adam M, van den Akker J, Perot C, Pellegrino B, Landman-Parker J, Douay L, Berger R, Bernard OA
Blood. 2002 ; 100 (3) : 991-997.
PMID 12130513
 
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed

Contributor(s)

Written02-2002Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France
Updated06-2002Roland Berger
EMI 0210, Faculté Necker Enfants-Malades, Paris, France

Citation

This paper should be referenced as such :
Berger, R
t(5;14)(q35;q32)
Atlas Genet Cytogenet Oncol Haematol. 2002;6(4):293-294.
Free journal version : [ pdf ]   [ DOI ]
History of this paper:
Berger, R. t(5;14)(q35;q32). Atlas Genet Cytogenet Oncol Haematol. 2002;6(4):293-294.
http://documents.irevues.inist.fr/bitstream/2042/37901/1/06-2002-t0514q35q32ID1227.pdf
URL : http://AtlasGeneticsOncology.org/Anomalies/t0514q35q32ID1227.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Tue Feb 17 19:03:05 CET 2015


Home   Genes   Leukemias   Solid Tumours   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

For comments and suggestions or contributions, please contact us

jlhuret@AtlasGeneticsOncology.org.