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t(9;11)(q34;p15) NUP98/PRRX2

Written2005-01Jean-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
ICD-Morpho 9861/3 AML with mutated NPM1; AML with mutated CEBPA; Acute myeloid leukaemia, NOS
Atlas_Id 1380

Clinics and Pathology

Disease Acute myelomonocytic leukemia (M4 AML)
Etiology This leukemia case is likely to be treatment related (a lymphoma was treated 4 years previously).
Epidemiology Only one case to date: a 65-year-old female patient.
Prognosis No data.

Genes involved and Proteins

Gene Name PRRX2
Location 9q34
Protein Class II homeobox gene.
Gene Name NUP98
Location 11p15.4
Protein Nucleoporin 98, a 98 kDa component of the nuclear pore complex implicated in nucleo-cytoplasmic transport.

Result of the chromosomal anomaly

Hybrid gene
 
Description 5'--> exon 11 of NUP98 is fused in frame with PRRX2 exon 2 to 3'.
  

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

A new translocation t(9;11)(q34;p15) fuses NUP98 to a novel homeobox partner gene, PRRX2, in a therapy-related acute myeloid leukemia.
Gervais C, Mauvieux L, Perrusson N, Hélias C, Struski S, Leymarie V, Lioure B, Lessard M
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 2005 ; 19 (1) : 145-148.
PMID 15496970
 

Citation

This paper should be referenced as such :
Huret, JL
t(9;11)(q34;p15)
Atlas Genet Cytogenet Oncol Haematol. 2005;9(1):32-33.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0911q34p15ID1380.html


Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes PRRX2

Translocations implicated (Data extracted from papers in the Atlas)

 t(9;11)(q34;p15) NUP98/PRRX2

External links

NUP98 (11p15.4) PRRX2 (9q34.11)

NUP98 (11p15.4) PRRX2 (9q34.11)

Mitelman databaset(9;11)(q34;p15) [Case List]    t(9;11)(q34;p15) [Association List] Mitelman database (CGAP - NCBI)
arrayMapTopo ( C42) Morph ( 9861/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
Mitelman databaseNUP98/PRRX2 [MCList]  NUP98 (11p15.4) PRRX2 (9q34.11)
TICdbNUP98/PRRX2  NUP98 (11p15.4) PRRX2 (9q34.11)
 
Disease databaset(9;11)(q34;p15) NUP98/PRRX2
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 11:23:32 CEST 2017


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