t(3;18)(q26;q11) ?/MECOM

Identity

ICD-Topo	C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho	9989/3 Myelodysplastic syndrome, unclassifiable
Atlas_Id	1283

Clinics and Pathology

Disease	Myelodysplastic syndrome
Epidemiology	Only one case to date, a 73 year old female patient
Prognosis	No data

Cytogenetics

Cytogenetics Morphological

There was also a del(5q)

Genes involved and Proteins

Note	The partner of EVI1 is yet unknown.

Gene Name	MECOM (Ecotropic Viral Integration Site 1 (EVI1) and Myelodysplastic Syndrome 1 (MDS1-EVI1)
Location	3q26.2
Protein	Transcrition factor; EVI1 targets include:GATA2, ZBTB16 /PLZF, ZFPM2/FOG2, JNK and the PI3K/AKT pathway. Role in cell cycle progression, likely to be cell-type dependant; antiapoptotic factor; involved in neuronal development organogenesis; role in hematopoietic differsntiation

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

EVI1 is consistently expressed as principal transcript in common and rare recurrent 3q26 rearrangements.

Poppe B, Dastugue N, Vandesompele J, Cauwelier B, De Smet B, Yigit N, De Paepe A, Cervera J, Recher C, De Mas V, Hagemeijer A, Speleman F

Genes, chromosomes & cancer. 2006 ; 45 (4) : 349-356.

PMID 16342172

The oncogene and developmental regulator EVI1: expression, biochemical properties, and biological functions.

Wieser R

Gene. 2007 ; 396 (2) : 346-357.

PMID 17507183

Citation

This paper should be referenced as such :

Huret, JL

t(3;18)(q26;q11)

Atlas Genet Cytogenet Oncol Haematol. 2008;12(4):349-349.

Free journal version : [ pdf ]   [ DOI ]

On line version : http://AtlasGeneticsOncology.org/Anomalies/t0318q26q11ID1283.html

Translocations implicated (Data extracted from papers in the Atlas)

	t(3;18)(q26;q11) ?/MECOM

External links

Mitelman database	t(3;18)(q26;q11) [Case List]    t(3;18)(q26;q11) [Association List] Mitelman database (CGAP - NCBI)
arrayMap	Topo ( C42) Morph ( 9989/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
Disease database	t(3;18)(q26;q11) ?/MECOM

REVIEW articles	automatic search in PubMed
Last year articles	automatic search in PubMed
All articles	automatic search in PubMed

© Atlas of Genetics and Cytogenetics in Oncology and Haematology

indexed on : Mon Sep 18 17:19:27 CEST 2017

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