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MECOM (Ecotropic Viral Integration Site 1 (EVI1) and Myelodysplastic Syndrome 1 (MDS1-EVI1)

Written2015-05Rotraud Wieser
Medizinische Universitaet Wien, Clinic of Medicine I, Waehringer Guertel 18-20, A-1090 Wien, Austria
This article is an update of :
2007-12Rotraud Wieser
Medizinische Universitaet Wien, Department fuer Medizinische Genetik, Waehringerstr. 10, A-1090 Wien, Austria
2003-05Soumen Chakraborty, Silvia Buonamici, Vitalyi Senyuk, Giuseppina Nucifora
Dept. of Pathology(Rm.3314), Molecular Biology, Research Building University Of Illinois At Chicago 900 South Ashland Avenue Chicago, IL-60607, USA

Abstract The MECOM locus in chromosome band 3q26.2 gives rise to two major mRNA and protein species, EVI1 and MDS1/EVI1, of which the former has been characterized far more extensively. EVI1 has been implicated in the maintenance and expansion of normal hematopoietic stem cells. In addition, it plays a role as an oncogene in myeloid leukemia and in certain solid tumors, its overexpression being associated with a dismal prognosis in several of these malignancies. EVI1 exerts its biological effects mainly by regulating gene transcription, and does so by acting as a sequence specific transcription factor, by modulating the activity of other sequence specific transcription factors, and by regulating promoter CpG island methylation.

Keywords Transcription regulation, myeloid leukemia, therapy resistance, hematopoietic stem cells

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Alias (NCBI)EVI1
HGNC Alias symbMDS1-EVI1
HGNC Alias namePR domain 3
HGNC Previous nameMDS1
HGNC Previous namemyelodysplasia syndrome 1
 ecotropic viral integration site 1
LocusID (NCBI) 2122
Atlas_Id 19
Location 3q26.2  [Link to chromosome band 3q26]
Location_base_pair Starts at 169083499 and ends at 169146305 bp from pter ( according to GRCh38/hg38-Dec_2013)  [Mapping MECOM.png]
  Genomic locus of the human EVI1 gene, and EVI1 and MDS1/EVI1 mRNA variants. Asterisk, translation initiation codon in EVI1 exon 3; diamond, translation stop codon in EVI1 exon 16. (This figure was reprinted from Gene 396, R. Wieser, 'The oncogene and developmental regulator EVI1: Expression, biochemical properties, and biological functions', pages 346-357, Copyright Elsevier (2007), with permission from Elsevier. Gene homepage:
  MECOM (Ecotropic Viral Integration Site 1 (EVI1) and Myelodysplastic Syndrome 1 (MDS1-EVI1) Hybridization with MECOM (EVI1) break apart probe (Kreatech, Leica Biosystems Inc., US) showing the gene on 3q26.2 (red-green or a fused yellow signal) - Courtesy Adriana Zamecnikova
Fusion genes
(updated 2017)
Data from Atlas, Mitelman, Cosmic Fusion, Fusion Cancer, TCGA fusion databases with official HUGO symbols (see references in chromosomal bands)
BCL11A (2p16.1)::MECOM (3q26.2)C2CD5 (12p12.1)::MECOM (3q26.2)CDK6 (7q21.2)::MECOM (3q26.2)
ETV6 (12p13.2)::MECOM (3q26.2)FNDC3B (3q26.31)::MECOM (3q26.2)HMCES (3q21.3)::MECOM (3q26.2)
HOXD1 (2q31.1)::MECOM (3q26.2)LINC01565 (3q21.3)::MECOM (3q26.2)MECOM (3q26.2)::CDK6 (7q21.2)
MECOM (3q26.2)::ETV6 (12p13.2)MECOM (3q26.2)::EYA3 (1p35.3)MECOM (3q26.2)::FRMD5 (15q15.3)
MECOM (3q26.2)::HOXD1 (2q31.1)MECOM (3q26.2)::LMAN2L (2q11.2)MECOM (3q26.2)::MECOM (3q26.2)
MECOM (3q26.2)::PRDM16 (1p36.32)MECOM (3q26.2)::PSMD2 (3q27.1)MECOM (3q26.2)::RAB7A (3q21.3)
MECOM (3q26.2)::RPL22 (1p36.31)MECOM (3q26.2)::RPN1 (3q21.3)MECOM (3q26.2)::RUNX1 (21q22.12)
MECOM (3q26.2)::SHISA5 (3p21.31)NRIP1 (21q11.2)::MECOM (3q26.2)PRDM16 (1p36.32)::MECOM (3q26.2)
PRKCI (3q26.2)::MECOM (3q26.2)PSMD2 (3q27.1)::MECOM (3q26.2)RPL22 (1p36.31)::MECOM (3q26.2)
RPN1 (3q21.3)::MECOM (3q26.2)RUNX1 (21q22.12)::MECOM (3q26.2)SKIL (3q26.2)::MECOM (3q26.2)
THADA (2p21)::MECOM (3q26.2)UBR2 (6p21.1)::MECOM (3q26.2)ZNF266 (19p13.2)::MECOM (3q26.2)


Description The human EVI1 gene spans approximately 65 kb of genomic DNA. 14 of its 16 exons are coding (Fig. 1A). Transcription can initiate from alternative exons 1a, 1b, 1c, 1d, or 3L (Fig. 1B), and several alternative splice variants of the EVI1 mRNA have been described (Delta324, -Rp9, Delta105; Fig. 1A).
The human gene consists of 4 exons spread over a genomic region of more than 500 kb. MDS1 exon 4 is located less than 2 kb upstream of EVI1 exon1a. MDS1 can also be expressed on its own. The MDS1/EVI1 mRNA presumably results from splicing of the second exon of MDS1 to the second exon of EVI1 (Fig. 1B).
Transcription Telomere to centromere.


  EVI1 and MDS1/EVI1 protein domains. Black boxes, zinc finger motifs; RD, repression domain; ac, acidic region; PR, PR domain. This figure was reprinted in modified form from Gene 396, R. Wieser, 'The oncogene and developmental regulator EVI1: Expression, biochemical properties, and biological functions', pages 346-357, Copyright Elsevier (2007), with permission from Elsevier. Gene homepage:
Description Exon 3 of the human EVI1 gene contains two closely spaced ATG codons, either of which may serve as the translation initiation site. Depending on which ATG is used, proteins of 1051 or 1041 amino acids will be formed. EVI1 contains two domains of seven and three zinc finger motifs, respectively, a repression domain between the two sets of zinc fingers, and an acidic domain at its C-terminus. It is a 145 kDa protein that is capable of binding to DNA in a sequence specific manner, and that interacts with transcriptional coactivators and corepressors as well as other sequence specific transcription factors. DNA binding and transcriptional regulation by EVI1 are influenced by posttranslational modifications like phosphorylation, acetylation, and sumoylation (Chakraborty et al, 2001; Shimahara et al, 2010; Bard-Chapeau et al, 2013; Singh et al, 2013; White et al, 2013).
Predicted translation of MDS1-EVI1 adds 188 amino acids to the N-terminus of EVI1. 63 of these additional amino acids are encoded by exon 2 and beginning of exon 3 of EVI1, and the remaining 125 from the MDS1 gene. MDS1-EVI1 contains a PR domain, which is about 40% homologous to the N-terminus of the retinoblastoma-binding protein, RIZ, and the PRDI-BF1 transcription factor. Some biological functions of MDS1/EVI1 were reported to be different from, or even antagonistic to, those of EVI1, while in other cases, EVI1 and MDS1/EVI1 acted in a similar manner. MDS1/EVI1 (PRDM3) has H3K9me1 methyltransferase activity and a role in maintaining heterochromatin integrity (Pinheiro et al, 2012).
Expression Among human tissues/organs, the EVI1 mRNA is expressed abundantly in kidney, lung, pancreas, stomach, ovaries, uterus, and prostate, to a lesser extent in the small intestine, colon, thymus, spleen, heart, brain, testis, and placenta, and at very low levels in skeletal muscle and bone marrow. The pattern of expression of MDS1-EVI1 is very similar to that of EVI1.
In the adult mouse, the Evi1 mRNA is expressed, at varying levels, in the kidney, lung, stomach, ovary, uterus, intestine, thymus, spleen, heart, brain, and liver. In the mouse embryo, Evi1 mRNA levels are high in the urinary system and Mullerian ducts, the lung, the heart, and the emerging limb buds.
Similar Evi1 expression patterns were also observed in Xenopus, chicken, and zebrafish.
In human and murine hematopoiesis, EVI1 mRNA levels are high in the most immature cell populations and decline in the course of differentiation (Kataoka et al, 2011; Bindels et al, 2012; Steinleitner et al, 2012).
EVI1 expression is regulated by RUNX1 and ELK1, by retinoic acid via RAR/RXR, and by certain MLL fusion proteins (Bingemann et al, 2009; Arai et al, 2011; Maicas et al, 2013).
Localisation Nuclear; in part in speckles.
Function Because of the spatially and temporally restricted expression of MECOM, it has been suggested that this gene plays important roles in development and could be involved in organogenesis, cell migration, cell growth, and differentiation.
In the mouse, homozygous disruption of the 6th exon of the Evi1 gene led to embryonic lethality, with widespread hypocellularity, reduced body size, small or absent limb buds, a pale yolk sac and placenta, abnormal development of the nervous system and the heart, and massive haemorrhaging. (Hoyt et al, 1997). Functions of Evi1 and/or Mds1/Evi1 in heart development, spine formation, and, particularly, maintenance and expansion of hematopoietic stem cells (HSCs) have been deduced from, or confirmed through, the phenotypes of additional MECOM knockout models (Goyama et al, 2008; Kataoka et al, 2011; Zhang et al, 2011; Bard-Chapeau et al, 2014; Juneja et al, 2014). A role of Evi1 in HSCs was also corroborated through overexpression and gene marking experiments (Buonamici et al, 2004; Laricchia-Robbio et al, 2008; Dickstein et al, 2010; Kataoka et al, 2011).
Support for a role of Evi1 as a leukemia initiating and promoting oncogene has been obtained through mouse bone marrow transduction/transplantation models, in which overexpression of Evi1 alone caused a myelodysplastic syndrome (MDS) like disease, while its co-expression with other oncogenes led to AML (Buonamici et al, 2004; Jin et al, 2007; Watanabe-Okochi et al, 2008; Watanabe-Okochi et al, 2013). Even more compellingly, in a human gene therapy trial for chronic granulomatous disease, activating integrations of the therapeutic vector into the MECOM locus led to clonal expansion with progression to MDS and, ultimately, AML (Stein et al, 2010). Evi1 was proposed to be essential for AML leukemia stem cell (LSC) function since its experimental down-regulation reduced leukemogenicity in several mouse models of AML (Goyama et al, 2008; Bindels et al, 2012). Evi1 expression was also associated with leukemia initiating capacity in chronic myeloid leukemia (CML) (Sato et al, 2014). A prominent role of EVI1 in therapy resistance was suggested by a number of clinical trials, and illustrated by in vitro data demonstrating that its ectopic expression reduced, and its knockdown enhanced, cellular responsiveness to chemotherapeutic drugs (Bindels et al, 2012; Konantz et al, 2012; Yamakawa et al, 2012; Rommer et al, 2013).
Beyond its roles in normal and malignant hematopoiesis, EVI1 negatively regulated NF-kB dependent inflammation (Xu et al, 2012) and promoted adipocyte differentiation (Ishibashi et al, 2012).
EVI1 exerts its biological functions mainly by acting as a transcription factor. and regulates the expression of both protein coding and miRNA genes. Reported direct EVI1 target genes are MS4A3 (Heller et al, 2015), PLZF (Takahashi and Licht, 2002), Gata2 (Yuasa et al; 2005), Pbx1 (Shimabe et al, 2009), Pten (Yoshimi et al, 2011), Gpr56 (Saito et al, 2013), DeltaNp63 (Nayak et al, 2013), Bcl-xL (Pradhan et al, 2011), Calreticulin (Qiu et al, 2008), Ppargamma2 (Ishibashi et al, 2012), miR-1-2 (Gomez-Benito et al, 2010), miR-9 (Senyuk et al, 2013), miR-124 (Dickstein et al, 2010), and miR-449A (De Weer et al, 2011). ChIP-seq, combined with genome wide gene expression profiling, has been employed for large-scale identification of EVI1 target genes in ovarian cancer and murine myeloid cell lines (Bard-Chapeau et al, 2012; Glass et al, 2013). EVI1 associates with a number of transcriptional cofactors like HDAC1 (Vinatzer et al, 2001), CtBP1 (Palmer et al, 2001), CtBP2 (Turner and Crossley, 1998), CBP, P/CAF (Chakraborty et al, 2001), the histone methyl transferases SUV39H1 and G9a (Spensberger et al, 2008a; Goyama et al, 2010), the ATP dependent helicases BRG1 and BRM (Chi et al, 2003), and the member of histone deacetylase complex, Mbd3b (Spensberger et al, 2008b). EVI1 was also shown to interact with DNA methyl transferases (Lugthart et al, 2011; Senyuk et al, 2011), leading to methylation of CpG islands of some of its target genes, among them, CADM1 (Fisser et al, 2014), miR-9 (Senyuk et al, 2013), and miR-124 (Dickstein et al, 2010). Furthermore, EVI1 interacted with, and modulated the function of, other sequence specific transcription factors, e.g. GATA1 (Laricchia-Robbio et al, 2006), RUNX1/AML1 (Senyuk et al, 2007), PU.1 (Laricchia-Robbio et al, 2009), SMAD3 (Kurokawa et al, 1998; Izutsu et al, 2001; Alliston et al, 2005), FOS (Bard-Chapeau et al, 2012), NFkB (Xu et al, 2012), and RAR/RXR (Bingemann et al, 2009; Steinmetz et al, 2014).
In addition to its activity as a regulator of transcription, EVI1 has been reported to inhibit c-jun N-terminal kinase (Kurokawa et al, 2000), and to stimulate PI3K/AKT signalling (Liu et al, 2006; Yoshimi et al, 2011).
Homology EVI1 orthologs are present in many species. EVI1 proteins from other mammals share more than 90% amino acid sequence identity with the human protein, and Xenopus EVI1 is 77% identical to its human counterpart. MDS1-EVI1 shares an overall homology with the C. elegans Egl 43 protein that includes the PR domain at the N-terminus and the two zinc-finger domains. An MDS1/EVI1 ortholog, hamlet, is also present in Drosophila.

Implicated in

Entity or
Note 3q21q26 syndrome. Chromosomal rearrangements located either 5' or 3' of the EVI1 gene can activate its transcription in haematopoietic cells. t(3;3)(q21;q26) breakpoints are often located 5' of EVI1, and inv(3)(q21q26) breakpoints 3' of it. In either case, transcription of EVI1 is activated by the distal enhancer of the GATA2 gene that is located in 3q21 (Gröschel et al, 2014; Yamazaki et al, 2014).
Disease Acute Myelogenous Leukemia (AML), Myelodysplastic Syndrome (MDS), and .
Prognosis Patients with EVI1 rearrangements have elevated platelet counts, marked hyperplasia with dysplasia of megakaryocytes, and a poor prognosis.
Cytogenetics Rearrangements at 3q26 may occur as a sole anomaly, but are often associated with monosomy 7 or deletion of the long arm of chromosome 7, and, less frequently, deletion in chromosome 5.
Normal and leukemia-associated EVI1 protein variants.
Oncogenesis Inappropriate expression of EVI1 in haematopoietic cells alters differentiation into granulocytes, erythrocytes and megakaryocytes. EVI1 promotes the proliferation of certain cell types, but inhibits the growth of others. It interferes with growth inhibition by TGF-b and with apoptosis elicited by a variety of stimuli. In a murine bone marrow transduction/transplantation model, experimental expression of EVI1 caused a disease resembling human myelodysplastic syndrome (Buonamici et al, 2004), while its coexpression with Hoxa9 and Meis 1, a mutated RUNX1 gene, or LIP led to overt leukemia (Jin et al, 2007; Watanabe-Okochi et al, 2008; Watanabe-Okochi et al, 2013).
Disease CML during the blast crisis and MDS in transformation.
Prognosis Poor.
Cytogenetics Complex.
Abnormal Protein Overexpression of a fusion protein between the amino terminus of ETV6, which does not contain any functional domains, and the entire MDS1/EVI1 protein is driven by the ETV6 promoter.
Disease Therapy-related MDS/AML and CML during the blast crisis.
Prognosis Poor.
Cytogenetics Complex.
Abnormal Protein RUNX1 /MDS1/EVI1
Oncogenesis RUNX1/MDS1/EVI1 is a chimeric transcription factor that interferes with RUNX1 functions in a dominant negative manner, but shares some biological effects with EVI1.
Entity Various cancers with known fusion genes
Cytogenetics Other translocations involving MECOM at 3q26 have been found:
THADA/MECOM in acute myeloid leukemia
BCL11A/MECOM in acute myeloid leukemia
MECOM/LMAN2L in ovarian carcinoma (Yoshihara et al., 2014)
LINC01565/MECOM in acute myeloid leukemia (Pekarsky et al., 1997)
MECOM/CDK6 in myeloid leukemias
TRB/MECOM in acute myeloid leukemia (M0-AML) (Suzukawa et al., 1999)
PVT1/MECOM in acute myeloid leukemia and myelodysplastic syndrome
MECOM/FRMD5 in lung adenocarcinoma (Yoshihara et al., 2014)
NRIP1/MECOM in acute myeloid leukemia (Haferlach et al., 2012)
Entity AML without 3q26 rearrangements.
Disease EVI1 may also be overexpressed in AML, MDS, or CML in blast crisis in the absence of any cytogenetically detectable 3q26 rearrangements.
Prognosis Poor (AML).
Entity Acute lymphoblastic leukemia
Note Overexpression of EVI1 reported in some cases (Konantz et al, 2013; Su et al, 2015)
Entity Chronic lymphoblastic leukemia
Prognosis Low EVI1 expression levels are associated with shorter survival (Vasyutina et al, 2015).
Entity Fanconi anemia
Note Overexpression of EVI1 (Meyer et al, 2007; Meyer et al, 2011)
Entity Ovarian cancer
Note Overexpression of EVI1
Prognosis Poor (Nanjundan et al, 2007)
Entity Estrogen receptor negative breast cancer
Note Overexpression of EVI1
Prognosis Poor (Patel et al, 2010)
Entity Infratentorial ependymoma
Note Overexpression of EVI1
Prognosis Poor (Koos et al, 2011)
Entity Pancreatic cancer
Note Overexpression of EVI1 (Tanaka et al, 2014)
Entity Colon cancer
Note Overexpression of EVI1 (Deng et al, 2013)




Repression of bone morphogenetic protein and activin-inducible transcription by Evi-1
Alliston T, Ko TC, Cao Y, Liang YY, Feng XH, Chang C, Derynck R
J Biol Chem 2005 Jun 24;280(25):24227-37
PMID 15849193
Conservation and expression of a novel alternatively spliced Evi1 exon
Alzuherri H, McGilvray R, Kilbey A, Bartholomew C
Gene 2006 Dec 15;384:154-62
PMID 17014970
Evi-1 is a transcriptional target of mixed-lineage leukemia oncoproteins in hematopoietic stem cells
Arai S, Yoshimi A, Shimabe M, Ichikawa M, Nakagawa M, Imai Y, Goyama S, Kurokawa M
Blood 2011 Jun 9;117(23):6304-14
PMID 21190993
Regulation of the expression of the oncogene EVI1 through the use of alternative mRNA 5'-ends
Aytekin M, Vinatzer U, Musteanu M, Raynaud S, Wieser R
Gene 2005 Aug 15;356:160-8
PMID 16014322
Mice carrying a hypomorphic Evi1 allele are embryonic viable but exhibit severe congenital heart defects
Bard-Chapeau EA, Szumska D, Jacob B, Chua BQ, Chatterjee GC, Zhang Y, Ward JM, Urun F, Kinameri E, Vincent SD, Ahmed S, Bhattacharya S, Osato M, Perkins AS, Moore AW, Jenkins NA, Copeland NG
PLoS One 2014 Feb 27;9(2):e89397
PMID 24586749
High EVI1 expression predicts poor survival in acute myeloid leukemia: a study of 319 de novo AML patients
Barjesteh van Waalwijk van Doorn-Khosrovani S, Erpelinck C, van Putten WL, Valk PJ, van der Poel-van de Luytgaarde S, Hack R, Slater R, Smit EM, Beverloo HB, Verhoef G, Verdonck LF, Ossenkoppele GJ, Sonneveld P, de Greef GE, Löwenberg B, Delwel R
Blood 2003 Feb 1;101(3):837-45
PMID 12393383
Induction of two alternatively spliced evi-1 proto-oncogene transcripts by cAMP in kidney cells
Bartholomew C, Clark AM
Oncogene 1994 Mar;9(3):939-42
PMID 8108138
EVI1 is critical for the pathogenesis of a subset of MLL-AF9-rearranged AMLs
Bindels EM, Havermans M, Lugthart S, Erpelinck C, Wocjtowicz E, Krivtsov AV, Rombouts E, Armstrong SA, Taskesen E, Haanstra JR, Beverloo HB, Döhner H, Hudson WA, Kersey JH, Delwel R, Kumar AR
Blood 2012 Jun 14;119(24):5838-49
PMID 22553314
Zinc finger transcription factor ecotropic viral integration site 1 is induced by all-trans retinoic acid (ATRA) and acts as a dual modulator of the ATRA response
Bingemann SC, Konrad TA, Wieser R
FEBS J 2009 Nov;276(22):6810-22
PMID 19843176
An interphase fluorescence in situ hybridisation assay for the detection of 3q26
Bobadilla D, Enriquez EL, Alvarez G, Gaytan P, Smith D, Slovak ML
2/EVI1 rearrangements in myeloid malignancies Br J Haematol
PMID 17341266
Alternative splicing of the Evi-1 zinc finger gene generates mRNAs which differ by the number of zinc finger motifs
Bordereaux D, Fichelson S, Tambourin P, Gisselbrecht S
Oncogene 1990 Jun;5(6):925-7
PMID 2113669
EVI1 induces myelodysplastic syndrome in mice
Buonamici S, Li D, Chi Y, Zhao R, Wang X, Brace L, Ni H, Saunthararajah Y, Nucifora G
J Clin Invest 2004 Sep;114(5):713-9
PMID 15343390
Interaction of EVI1 with cAMP-responsive element-binding protein-binding protein (CBP) and p300/CBP-associated factor (P/CAF) results in reversible acetylation of EVI1 and in co-localization in nuclear speckles
Chakraborty S, Senyuk V, Sitailo S, Chi Y, Nucifora G
J Biol Chem 2001 Nov 30;276(48):44936-43
PMID 11568182
EVI1 promotes cell proliferation by interacting with BRG1 and blocking the repression of BRG1 on E2F1 activity
Chi Y, Senyuk V, Chakraborty S, Nucifora G
J Biol Chem 2003 Dec 12;278(50):49806-11
PMID 14555651
EVI1-mediated down regulation of MIR449A is essential for the survival of EVI1 positive leukaemic cells
De Weer A, Van der Meulen J, Rondou P, Taghon T, Konrad TA, De Preter K, Mestdagh P, Van Maerken T, Van Roy N, Jeison M, Yaniv I, Cauwelier B, Noens L, Poirel HA, Vandenberghe P, Lambert F, De Paepe A, Sánchez MG, Odero M, Verhasselt B, Philippé J, Vandesompele J, Wieser R, Dastugue N, Van Vlierberghe P, Poppe B, Speleman F
Br J Haematol 2011 Aug;154(3):337-48
PMID 21569010
Overexpression of Evi-1 oncoprotein represses TGF- signaling in colorectal cancer
Deng X, Cao Y, Liu Y, Li F, Sambandam K, Rajaraman S, Perkins AS, Fields AP, Hellmich MR, Townsend CM Jr, Thompson EA, Ko TC
Mol Carcinog 2013 Apr;52(4):255-64
PMID 22161860
Methylation and silencing of miRNA-124 by EVI1 and self-renewal exhaustion of hematopoietic stem cells in murine myelodysplastic syndrome
Dickstein J, Senyuk V, Premanand K, Laricchia-Robbio L, Xu P, Cattaneo F, Fazzina R, Nucifora G
Proc Natl Acad Sci U S A 2010 May 25;107(21):9783-8
PMID 20448201
Insertional mutagenesis identifies genes that promote the immortalization of primary bone marrow progenitor cells
Du Y, Jenkins NA, Copeland NG
Blood 2005 Dec 1;106(12):3932-9
PMID 16109773
Induction of the proapoptotic tumor suppressor gene Cell Adhesion Molecule 1 by chemotherapeutic agents is repressed in therapy resistant acute myeloid leukemia
Fisser MC, Rommer A, Steinleitner K, Heller G, Herbst F, Wiese M, Glimm H, Sill H, Wieser R
Mol Carcinog 2014 Dec 9
PMID 25491945
EVI1 controls proliferation in acute myeloid leukaemia through modulation of miR-1-2
Gómez-Benito M, Conchillo A, García MA, Vázquez I, Maicas M, Vicente C, Cristobal I, Marcotegui N, García-Ortí L, Bandrés E, Calasanz MJ, Alonso MM, Odero MD
Br J Cancer 2010 Oct 12;103(8):1292-6
PMID 20842122
Global Identification of EVI1 Target Genes in Acute Myeloid Leukemia
Glass C, Wuertzer C, Cui X, Bi Y, Davuluri R, Xiao YY, Wilson M, Owens K, Zhang Y, Perkins A
PLoS One 2013 Jun 27;8(6):e67134
PMID 23826213
EVI-1 interacts with histone methyltransferases SUV39H1 and G9a for transcriptional repression and bone marrow immortalization
Goyama S, Nitta E, Yoshino T, Kako S, Watanabe-Okochi N, Shimabe M, Imai Y, Takahashi K, Kurokawa M
Leukemia 2010 Jan;24(1):81-8
PMID 19776757
Evi-1 is a critical regulator for hematopoietic stem cells and transformed leukemic cells
Goyama S, Yamamoto G, Shimabe M, Sato T, Ichikawa M, Ogawa S, Chiba S, Kurokawa M
Cell Stem Cell 2008 Aug 7;3(2):207-20
PMID 18682242
A single oncogenic enhancer rearrangement causes concomitant EVI1 and GATA2 deregulation in leukemia
Gröschel S, Sanders MA, Hoogenboezem R, de Wit E, Bouwman BA, Erpelinck C, van der Velden VH, Havermans M, Avellino R, van Lom K, Rombouts EJ, van Duin M, Döhner K, Beverloo HB, Bradner JE, Döhner H, Löwenberg B, Valk PJ, Bindels EM, de Laat W, Delwel R
Cell 2014 Apr 10;157(2):369-81
PMID 24703711
Expression and prognostic significance of different mRNA 5'-end variants of the oncogene EVI1 in 266 patients with de novo AML: EVI1 and MDS1/EVI1 overexpression both predict short remission duration
Haas K, Kundi M, Sperr WR, Esterbauer H, Ludwig WD, Ratei R, Koller E, Gruener H, Sauerland C, Fonatsch C, Valent P, Wieser R
Genes Chromosomes Cancer 2008 Apr;47(4):288-98
PMID 18181178
Three novel cytogenetically cryptic EVI1 rearrangements associated with increased EVI1 expression and poor prognosis identified in 27 acute myeloid leukemia cases
Haferlach C, Bacher U, Grossmann V, Schindela S, Zenger M, Kohlmann A, Kern W, Haferlach T, Schnittger S
Genes Chromosomes Cancer 2012 Dec;51(12):1079-85
PMID 22887804
EVI1 promotes tumor growth via transcriptional repression of MS4A3
Heller G, Rommer A, Steinleitner K, Etzler J, Hackl H, Heffeter P, Tomasich E, Filipits M, Steinmetz B, Topakian T, Klingenbrunner S, Ziegler B, Spittler A, Zöchbauer-Müller S, Berger W, Wieser R
J Hematol Oncol 2015 Mar 21;8:28
PMID 25886616
The Evi1 proto-oncogene is required at midgestation for neural, heart, and paraxial mesenchyme development
Hoyt PR, Bartholomew C, Davis AJ, Yutzey K, Gamer LW, Potter SS, Ihle JN, Mucenski ML
Mech Dev 1997 Jul;65(1-2):55-70
PMID 9256345
An Evi1-C/EBP complex controls peroxisome proliferator-activated receptor 2 gene expression to initiate white fat cell differentiation
Ishibashi J, Firtina Z, Rajakumari S, Wood KH, Conroe HM, Steger DJ, Seale P
Mol Cell Biol 2012 Jun;32(12):2289-99
PMID 22473998
The EVI-1 gene--its role in pathogenesis of human leukemias
J J, Witt M
Leuk Res 2000 Jul;24(7):553-8
PMID 10867128
Trib1 and Evi1 cooperate with Hoxa and Meis1 in myeloid leukemogenesis
Jin G, Yamazaki Y, Takuwa M, Takahara T, Kaneko K, Kuwata T, Miyata S, Nakamura T
Blood 2007 May 1;109(9):3998-4005
PMID 17227832
Deletion of Mecom in mouse results in early-onset spinal deformity and osteopenia
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This paper should be referenced as such :
Rotraud Wieser
MECOM (Ecotropic Viral Integration Site 1 (EVI1) and Myelodysplastic Syndrome 1 (MDS1-EVI1)
Atlas Genet Cytogenet Oncol Haematol. 2016;20(4):185-194.
Free journal version : [ pdf ]   [ DOI ]
History of this paper:
Chakraborty, S ; Buonamici, S ; Senyuk, V ; Nucifora, G. EVI1-MDS1/EVI1 (ecotropic viral integration site 1 (EVI1), myelodysplastic syndrome 1 (MDS1)-EVI1). Atlas Genet Cytogenet Oncol Haematol. 2003;7(3):160-161.
Wieser, R. EVI1 (ecotropic viral integration site 1 (EVI1), myelodysplastic syndrome 1 (MDS1)-EVI1). Atlas Genet Cytogenet Oncol Haematol. 2008;12(4):306-310.

Other Leukemias implicated (Data extracted from papers in the Atlas) [ 42 ]
  21q22 rearrangements (RUNX1) in treatment related leukemia
3q21q26 rearrangements (RPN1::MECOM) in treatment related leukemia
3q26 rearrangements (MECOM) in myeloid malignancies
Acute myeloid leukemia with myelodysplasia related changes
Classification of myelodysplastic syndromes 2015
Classification of myelodysplasic syndromes 1999
Chronic myelogenous leukaemia (CML)
TBL1XR1::MECOM fusion::del(3)(q26.2q26.3) TBL1XR1/MECOM
i(3)(q10) in non-Hodgkin's lymphoma (NHL)
inv(3)(q21q26) RPN1::MECOM::t(3;3)(q21;q26) RPN1::MECOM::ins(3;3)(q26;q21q26) RPN1::MECOM
inv(3)(p12q26) ?::MECOM
inv(3)(p24q26) ?::MECOM
inv(3)(q21q26) RPN1::MECOM
inv(3)(q23q26) ?::MECOM
t(1;3)(p36;q21) PSMD2::PRDM16 ???
t(2;3)(p16;q26) BCL11A::MECOM
t(2;3)(p21;q26) THADA::MECOM
t(2;3)(p15-23;q26-27) ?::MECOM
t(3;3)(p24;q26) ?::MECOM
t(3;5)(q26;q31) H2AFY::MECOM
t(3;5)(q26;q34) ?::MECOM
t(3;6)(q25;q26) ?::MECOM
t(3;7)(q26;q21) MECOM::CDK6
t(3;7)(q26;q21) CDK6::MECOM
t(3;8)(q26;q24) PVT1::MECOM
t(3;9)(q26;p23) ?::MECOM
t(3;11)(q26;p15) ?::MECOM
t(3;12)(q26;p13) ETV6::MECOM::t(3;12)(q26;p13) ETV6/EVI1
t(3;12)(q26;q21) ?::MECOM
t(3;17)(q26;q22) ?::MECOM
t(3;18)(q26;q11) ?::MECOM
t(3;21)(q26;q22) RUNX1::MECOM
t(3;21)(q26;q11) NRIP1::MECOM
t(3;21)(q26;q22) RUNX1::MECOM in treatment related leukemia
t(6;11)(q27;q23) KMT2A::AFDN
t(9;13)(p12;q21) PAX5::DACH1
Therapy-Related Hematopoietic Neoplasia
ins(3;7)(q26;q34q22) TRB::MECOM
t(3;3)(q26;q21) LINC01565::MECOM
inv(3)(q21q26):t(3;3)(q21;q26):t(3;3)(q26;q21) GATA2::MECOM
t(3;3)(q26;q26) FNDC3B::MECOM
t(3;7)(q26;q34) MECOM::TCRB1

External links


HGNC (Hugo)MECOM   3498
LRG (Locus Reference Genomic)LRG_1118
Entrez_Gene (NCBI)MECOM    MDS1 and EVI1 complex locus
AliasesAML1-EVI-1; EVI1; KMT8E; MDS1; 
GeneCards (Weizmann)MECOM
Ensembl hg19 (Hinxton)ENSG00000085276 [Gene_View]
Ensembl hg38 (Hinxton)ENSG00000085276 [Gene_View]  ENSG00000085276 [Sequence]  chr3:169083499-169146305 [Contig_View]  MECOM [Vega]
ICGC DataPortalENSG00000085276
Genatlas (Paris)MECOM
Genetics Home Reference (NIH)MECOM
Genomic and cartography
GoldenPath hg38 (UCSC)MECOM  -     chr3:169083499-169146305 -  3q26.2   [Description]    (hg38-Dec_2013)
GoldenPath hg19 (UCSC)MECOM  -     3q26.2   [Description]    (hg19-Feb_2009)
GoldenPathMECOM - 3q26.2 [CytoView hg19]  MECOM - 3q26.2 [CytoView hg38]
Genome Data Viewer NCBIMECOM [Mapview hg19]  
OMIM165215   616738   
Gene and transcription
Genbank (Entrez)AA043944 AF164154 AF164155 AF164156 AF164157
RefSeq transcript (Entrez)NM_001105077 NM_001105078 NM_001163999 NM_001164000 NM_001205194 NM_001366466 NM_001366467 NM_001366468 NM_001366469 NM_001366470 NM_001366471 NM_001366472 NM_001366473 NM_001366474 NM_004991 NM_005241
Consensus coding sequences : CCDS (NCBI)MECOM
Gene Expression Viewer (FireBrowse)MECOM [ Firebrowse - Broad ]
GenevisibleExpression of MECOM in : [tissues]  [cell-lines]  [cancer]  [perturbations]  
BioGPS (Tissue expression)2122
GTEX Portal (Tissue expression)MECOM
Human Protein AtlasENSG00000085276-MECOM [pathology]   [cell]   [tissue]
Protein : pattern, domain, 3D structure
UniProt/SwissProtQ13465   [function]  [subcellular_location]  [family_and_domains]  [pathology_and_biotech]  [ptm_processing]  [expression]  [interaction]
NextProtQ13465  [Sequence]  [Exons]  [Medical]  [Publications]
With graphics : InterProQ13465
Domaine pattern : Prosite (Expaxy)SET (PS50280)    ZINC_FINGER_C2H2_1 (PS00028)    ZINC_FINGER_C2H2_2 (PS50157)   
Domains : Interpro (EBI)SET_dom    Znf_C2H2_sf    Znf_C2H2_type   
Domain families : Pfam (Sanger)zf-C2H2 (PF00096)   
Domain families : Pfam (NCBI)pfam00096   
Domain families : Smart (EMBL)SET (SM00317)  ZnF_C2H2 (SM00355)  
Conserved Domain (NCBI)MECOM
PDB Europe6BW3   
PDB (PDBSum)6BW3   
PDB (IMB)6BW3   
Structural Biology KnowledgeBase6BW3   
SCOP (Structural Classification of Proteins)6BW3   
CATH (Classification of proteins structures)6BW3   
AlphaFold pdb e-kbQ13465   
Human Protein Atlas [tissue]ENSG00000085276-MECOM [tissue]
Protein Interaction databases
IntAct (EBI)Q13465
Ontologies - Pathways
Ontology : AmiGORNA polymerase II cis-regulatory region sequence-specific DNA binding  DNA-binding transcription activator activity, RNA polymerase II-specific  DNA binding  DNA-binding transcription factor activity  protein binding  nucleus  nucleus  nucleoplasm  cytosol  regulation of transcription by RNA polymerase II  apoptotic process  nuclear speck  cell differentiation  negative regulation of programmed cell death  negative regulation of transcription, DNA-templated  positive regulation of transcription, DNA-templated  positive regulation of transcription by RNA polymerase II  negative regulation of JNK cascade  metal ion binding  histone methyltransferase activity (H3-K9 specific)  histone H3-K9 methylation  regulation of cell cycle  heterochromatin organization  hematopoietic stem cell proliferation  
Ontology : EGO-EBIRNA polymerase II cis-regulatory region sequence-specific DNA binding  DNA-binding transcription activator activity, RNA polymerase II-specific  DNA binding  DNA-binding transcription factor activity  protein binding  nucleus  nucleus  nucleoplasm  cytosol  regulation of transcription by RNA polymerase II  apoptotic process  nuclear speck  cell differentiation  negative regulation of programmed cell death  negative regulation of transcription, DNA-templated  positive regulation of transcription, DNA-templated  positive regulation of transcription by RNA polymerase II  negative regulation of JNK cascade  metal ion binding  histone methyltransferase activity (H3-K9 specific)  histone H3-K9 methylation  regulation of cell cycle  heterochromatin organization  hematopoietic stem cell proliferation  
Pathways : KEGGMAPK signaling pathway    Pathways in cancer    Chronic myeloid leukemia   
Atlas of Cancer Signalling NetworkMECOM
Wikipedia pathwaysMECOM
Orthology - Evolution
GeneTree (enSembl)ENSG00000085276
Phylogenetic Trees/Animal Genes : TreeFamMECOM
Homologs : HomoloGeneMECOM
Homology/Alignments : Family Browser (UCSC)MECOM
Gene fusions - Rearrangements
Fusion : MitelmanETV6::MECOM [12p13.2/3q26.2]  
Fusion : MitelmanLINC01565::MECOM [3q21.3/3q26.2]  
Fusion : MitelmanMECOM::CDK6 [3q26.2/7q21.2]  
Fusion : MitelmanMECOM::FRMD5 [3q26.2/15q15.3]  
Fusion : MitelmanMECOM::LMAN2L [3q26.2/2q11.2]  
Fusion : MitelmanMECOM::RPN1 [3q26.2/3q21.3]  
Fusion : MitelmanNRIP1::MECOM [21q11.2/3q26.2]  
Fusion : MitelmanRPN1::MECOM [3q21.3/3q26.2]  
Fusion : MitelmanRUNX1::MECOM [21q22.12/3q26.2]  
Fusion : MitelmanTRB::MECOM [-/3q26.2]  
Fusion : QuiverMECOM
Polymorphisms : SNP and Copy number variants
NCBI Variation ViewerMECOM [hg38]
dbSNP Single Nucleotide Polymorphism (NCBI)MECOM
Exome Variant ServerMECOM
GNOMAD BrowserENSG00000085276
Varsome BrowserMECOM
ACMGMECOM variants
Genomic Variants (DGV)MECOM [DGVbeta]
DECIPHERMECOM [patients]   [syndromes]   [variants]   [genes]  
CONAN: Copy Number AnalysisMECOM 
ICGC Data PortalMECOM 
TCGA Data PortalMECOM 
Broad Tumor PortalMECOM
OASIS PortalMECOM [ Somatic mutations - Copy number]
Cancer Gene: CensusMECOM 
Somatic Mutations in Cancer : COSMICMECOM  [overview]  [genome browser]  [tissue]  [distribution]  
Somatic Mutations in Cancer : COSMIC3DMECOM
Mutations and Diseases : HGMDMECOM
intOGen PortalMECOM
LOVD (Leiden Open Variation Database)[gene] [transcripts] [variants]
DgiDB (Drug Gene Interaction Database)MECOM
DoCM (Curated mutations)MECOM
CIViC (Clinical Interpretations of Variants in Cancer)MECOM
Impact of mutations[PolyPhen2] [Provean] [Buck Institute : MutDB] [Mutation Assessor] [Mutanalyser]
OMIM165215    616738   
Orphanet22871    11011    10703   
Genetic Testing Registry MECOM
NextProtQ13465 [Medical]
Target ValidationMECOM
Huge Navigator MECOM [HugePedia]
Clinical trials, drugs, therapy
Protein Interactions : CTDMECOM
Pharm GKB GenePA27912
Clinical trialMECOM
DataMed IndexMECOM
PubMed205 Pubmed reference(s) in Entrez
GeneRIFsGene References Into Functions (Entrez)
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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indexed on : Fri Oct 8 21:17:32 CEST 2021

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