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t(10;11)(p12;q23) KMT2A/MLLT10

Written1997-12Jean-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 9807/3 Mixed phenotype acute leukaemia with t(v;11q23); MLL rearranged
ICD-Morpho 9811/3 B lymphoblastic leukaemia/lymphoma, NOS
ICD-Morpho 9813/3 B lymphoblastic leukaemia/lymphoma with t(v;11q23); MLL rearranged
ICD-Morpho 9861/3 AML with mutated NPM1; AML with mutated CEBPA; Acute myeloid leukaemia, NOS
ICD-Morpho 9920/3 Therapy-related myeloid neoplasms
Atlas_Id 1025

Clinics and Pathology

Disease AML
Phenotype / cell stem origin M5/M4; ALL at times, therapy related cases
Epidemiology from infants to adults
Prognosis poor

Cytogenetics

Cytogenetics Morphological A high diversity of reported breakpoints on chromosome 10, from 10p11 to 10p15: remains to be fully studiedL
Additional anomalies inv(11)(q13q23) is frequently acompanying the t(10;11); this is likely to be related to the transcription directions of the two genes

Genes involved and Proteins

Gene Name MLLT10
Location 10p12
Dna / Rna 5' telomeric --> 3' centromeric orientation
Protein contains 3 Zn fingezrs and a leucine zipper; nuclear localisation; transcription factor
Gene Name KMT2A
Location 11q23
Dna / Rna 21 exons, spanning over 100 kb; 13-15 kb mRNA
Protein 431 kDa; contains two DNA binding motifs (a AT hook, and Zinc fingers), a DNA methyl transferase motif, a bromodomain; transcriptional regulatory factor; nuclear localisation

Result of the chromosomal anomaly

Hybrid gene
Description 5' MLL - 3' AF10; variable breakpoints on both chromosomes; the fusion transcript on chromosome 11 is the crucial one; MLL and AF10 are in opposite transcriptional directions, wich may be cause of the complexity of the translocation
  
Fusion Protein
Description N-term AT hook and DNA methyltransferase from MLL fused to the leucine zipper C-term of AF10
Expression Localisation nuclear localisation
  

To be noted

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

Bibliography

The t(10;11) translocation in acute myeloid leukemia (M5) consistently fuses the leucine zipper motif of AF10 onto the HRX gene.
Chaplin T, Bernard O, Beverloo HB, Saha V, Hagemeijer A, Berger R, Young BD
Blood. 1995 ; 86 (6) : 2073-2076.
PMID 7662954
 

Citation

This paper should be referenced as such :
Huret, JL
t(11;19)(q23;p13.1)
Atlas Genet Cytogenet Oncol Haematol. 1997;1(2):102-103.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t1011ID1025.html


Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes MLLT10 KMT2A

Translocations implicated (Data extracted from papers in the Atlas)

 t(10;11)(p12;q23) KMT2A/MLLT10

External links

KMT2A (11q23.3) MLLT10 (10p12.31)

KMT2A (11q23.3) MLLT10 (10p12.31)

Mitelman databaset(10;11)(p12;q23) [Case List]    t(10;11)(p12;q23) [Association List] Mitelman database (CGAP - NCBI)
arrayMapTopo ( C42) Morph ( 9807/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9811/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9813/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9861/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9920/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
COSMIC_fusionKMT2A/MLLT10 KMT2A (11q23.3) MLLT10 (10p12.31)   [fusion2033]   [fusion2034]   [fusion2035]   [fusion2036]   [fusion2037]   [fusion2038]   [fusion2039]   [fusion2040]   [fusion2041]   [fusion2042]   [fusion2043]   [fusion2044]   [fusion2045]   [fusion2046]   [fusion2047]   [fusion2048]   [fusion2049]   [fusion2050]   [fusion2051]   [fusion2052]  
TICdbKMT2A/MLLT10  KMT2A (11q23.3) MLLT10 (10p12.31)
 
Disease databaset(10;11)(p12;q23) KMT2A/MLLT10
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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