t(3;11)(q28;q23) KMT2A/LPP

2001-10-01   Jean-Loup Huret  

1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

Clinics and Pathology

Disease

treatment related acute myeloid leukemia (t-AML) so far

Epidemiology

only 1 case available

Clinics

a 41 yr old female patient with a previous history of non Hodgkin lymphoma treated withtopoisomerase II inhibitors 3 yrs earlier; she developped a M5a t-AML and died 10 days after diagnosis

Genes Involved and Proteins

Gene name
LPP (lipoma preferred partner)
Location
3q28
Protein description
LPP is a member of the LIM protein gene family, containing a leucin-zipper motif in its amino-terminal region and three LIM domains in its carboxy-terminal region
Gene name
KMT2A (myeloid/lymphoid or mixed lineage leukemia)
Location
11q23.3
Protein description
contains two DNA binding motifs (a AT hook, and Zinc fingers), a DNA methyl transferase motif, a bromodomain; transcriptional regulatory factor involved in maintenance of Hox gene expression

Result of the Chromosomal Anomaly

Description

5 MLL including the 8 first exons fused to the 3 last 3 exons (exons 9 to 11) of LPP.

Transcript

the reciprocal 5MLPP-3MLL is also transcribed

Description

N-term AT hook and DNA methyltransferase from MLL fused to the 2 last C-term LIM domains of LPP

Article Bibliography

Pubmed IDLast YearTitleAuthors
114335292001Human LPP gene is fused to MLL in a secondary acute leukemia with a t(3;11) (q28;q23).Dahéron L et al

Summary

Fusion gene

KMT2A/LPP KMT2A (11q23.3) LPP (3q28) TIC

Citation

Jean-Loup Huret

t(3;11)(q28;q23) KMT2A/LPP

Atlas Genet Cytogenet Oncol Haematol. 2001-10-01

Online version: http://atlasgeneticsoncology.org/haematological/1223/t(3;11)(q28;q23)-kmt2a-lpp