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t(9;11)(q34;q23) FNBP1/KMT2A

Written2001-06Uta Fuchs, Arndt Borkhardt
Children's University Hospital Giessen, Hematology &Oncology, Feulgenstr. 12, 35392 Giessen, Germany

(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 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
Atlas_Id 1205
Other namesins(11;9)(q23;q34)inv(11)(q13;q23)

Clinics and Pathology

Disease acute myeloid leukemia (AML)
Phenotype / cell stem origin one case of M4-AML
Epidemiology unknown
Clinics WBC 35 X 109/l
Prognosis poor

Cytogenetics

Cytogenetics Molecular YAC 13HH4; P1 ICRFP700L11103Q5

Genes involved and Proteins

Gene NameFNBP1 (Formin Binding Protein 17)
Location 9q34.11
Dna / Rna at least 2042 bp
Protein at least 679 amino acids
Gene NameKMT2A (myeloid/lymphoid or mixed lineage leukemia)
Location 11q23.3
Dna / Rna 11910 bp
Protein 3970 amino acids; 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/FBP17-3'
  
Fusion Protein
 
Description Hybrid transcript MLL/FBP17 contains the following domains: from MLL: AT-hook, DNA-Methyltransferase, Zinc-Fingers; from FBP17:cdc15 homology region, putative rho-binding domain, SH3-domain
Expression Localisation nuclear
Oncogenesis unknown
  

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

The human formin-binding protein 17 (FBP17) interacts with sorting nexin, SNX2, and is an MLL-fusion partner in acute myelogeneous leukemia.
Fuchs U, Rehkamp G, Haas OA, Slany R, König M, Bojesen S, Bohle RM, Damm-Welk C, Ludwig WD, Harbott J, Borkhardt A
Proceedings of the National Academy of Sciences of the United States of America. 2001 ; 98 (15) : 8756-8761.
PMID 11438682
 

Citation

This paper should be referenced as such :
Fuchs, U ; Borkhardt, A
t(9;11)(q34;q23) FBP17/MLL
Atlas Genet Cytogenet Oncol Haematol. 2001;5(3):200-201.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0911FBPID1205.html


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

Genes DAB2IP FNBP1

Translocations implicated (Data extracted from papers in the Atlas)

 t(9;11)(q34;q23) FNBP1/KMT2A

External links

Mitelman databaset(9;11)(q34;q23) [Case List]    t(9;11)(q34;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 ( 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]
 
 
Disease databaset(9;11)(q34;q23) FNBP1/KMT2A
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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