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t(6;11)(q27;q23)

Identity

 
  t(6;11)(q27;q23) G- banding - Courtesy Diane H. Norback, Eric B. Johnson, Sara Morrison-Delap Cytogenetics at theWaisman Center (top and middle ), and Jean-Luc Lai (below)

Clinics and Pathology

Disease ANLL
Phenotype / cell stem origin M4, M5 mostly; T-cell ALL at times; therapy related AL
Epidemiology still poorly known; found in children and young adults; male predominance
Clinics frequent infections; organomegaly; blood data: moderate WBC
Treatment BMT is indicated
Prognosis very poor (as in other 11q23 rearrangements); rare remission; short survival

Cytogenetics

Cytogenetics Morphological may be missinterpreted as a del(11q), as chromosome 6 involvempent may be overlooked
Cytogenetics Molecular ... therefore, FISH may be needed
Additional anomalies are present in most cases; +8 in particular; +3; +19; +21

Genes involved and Proteins

Gene Name AF6
Location 6q27
Protein contains a GLGF motif; widely expressed; cytoplasmic localisation; role in signal transduction
Gene Name MLL
Location 11q23
Protein 431 KDa; contains two DNA binding motifs (a AT hook and Zinc fingers), and a DNA methyl transferase motif; wide expression; nuclear localisation; transcriptional regulatory factor

Result of the chromosomal anomaly

Hybrid gene
Description 5' MLL-3'AF6
  
Fusion Protein
Description about 1400 NH2-term amino acids (with the AT hook and DNA binding motifs) from MLL and most of AF6, starting at amino acid 26
  

External links

Other databaset(6;11)(q27;q23) Mitelman database (CGAP - NCBI)
Other databaset(6;11)(q27;q23) CancerChromosomes (NCBI)

To be noted

having a glance at 11q23 rearrangements, gives an overview of related diseases

Bibliography

Cloning of the ALL-1 fusion partner, the AF-6 gene, involved in acute myeloid leukemias with the t(6;11) chromosome translocation.
Prasad R, Gu Y, Alder H, Nakamura T, Canaani O, Saito H, Huebner K, Gale RP, Nowell PC, Kuriyama K
Cancer research. 1993 ; 53 (23) : 5624-5628.
PMID 8242616
 
Unique clinical features and prognostic significance of the translocation (6;11) in acute leukemia.
Welborn JL, Jenks HM, Hagemeijer A
Cancer genetics and cytogenetics. 1993 ; 65 (2) : 125-129.
PMID 8453597
 

Contributor(s)

Written11-1997Jean-Loup Huret

Citation

This paper should be referenced as such :
Huret JL . t(6;11)(q27;q23). Atlas Genet Cytogenet Oncol Haematol. November 1997 .
URL : http://AtlasGeneticsOncology.org/Anomalies/t0611.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Mon May 12 18:12:29 2008


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