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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 NamePRRX2 (paired related homeobox 2)
Location 9q34.11
Protein Class II homeobox gene.
Gene NameNUP98 (nucleoporin 98 kDa)
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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