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    CBFA2T3 CBFA2/RUNX1 partner transcriptional co-repressor 3 [ Homo sapiens (human) ]

    Gene ID: 863, updated on 10-Oct-2024

    GeneRIFs: Gene References Into Functions

    GeneRIFPubMed TitleDate
    CNS erythroblastic sarcoma: a potential emerging pediatric tumor type characterized by NFIA::RUNX1T1/3 fusions.

    CNS erythroblastic sarcoma: a potential emerging pediatric tumor type characterized by NFIA::RUNX1T1/3 fusions.
    Tauziède-Espariat A, Lew-Derivry L, Abbou S, Métais A, Pierron G, Reynaud S, Masliah-Planchon J, Mariet C, Hasty L, Dangouloff-Ros V, Boddaert N, Csanyi M, Aline-Fardin A, Lamaison C, Chrétien F, Beccaria K, Puget S, Varlet P., Free PMC Article

    02/1/2024
    The transcriptional corepressor CBFA2T3 inhibits all-trans-retinoic acid-induced myeloid gene expression and differentiation in acute myeloid leukemia.

    The transcriptional corepressor CBFA2T3 inhibits all-trans-retinoic acid-induced myeloid gene expression and differentiation in acute myeloid leukemia.
    Steinauer N, Guo C, Zhang J., Free PMC Article

    01/16/2021
    Embryonic erythropoiesis and hemoglobin switching require transcriptional repressor ETO2 to modulate chromatin organization.

    Embryonic erythropoiesis and hemoglobin switching require transcriptional repressor ETO2 to modulate chromatin organization.
    Guo X, Plank-Bazinet J, Krivega I, Dale RK, Dean A., Free PMC Article

    11/21/2020
    These findings suggest rs13333659 in CBFA2T3 as a risk locus to modulate the decline rate of cerebrospinal fluid amyloid-beta42 peptide preceding the onset of clinical symptoms

    Genome-wide association study identifies CBFA2T3 affecting the rate of CSF Aβ(42) decline in non-demented elders.
    Dou KX, Zhang C, Tan CC, Xu W, Li JQ, Cao XP, Tan L, Yu JT, Alzheimer’s Disease Neuroimaging Initiative (ADNI)., Free PMC Article

    08/15/2020
    CBFA2T3 is functional in t(8;21) acute myeloid leukemia cells, a possible RUNX1-RUNX1T1-mediated direct interference of CBFA2T3 corepressor function may be compensated by a higher threshold level of CBFA2T3 expression that is needed to drive the relapse of t(8;21) acute myeloid leukemia.

    Myeloid translocation gene CBFA2T3 directs a relapse gene program and determines patient-specific outcomes in AML.
    Steinauer N, Guo C, Huang C, Wong M, Tu Y, Freter CE, Zhang J., Free PMC Article

    05/16/2020
    Kaiso modifies MTG16-driven inflammation and tumorigenesis; this suggests that Kaiso deregulation contributes to MTG16-dependent colitis and Colitis-associated carcinoma phenotypes.

    Kaiso is required for MTG16-dependent effects on colitis-associated carcinoma.
    Short SP, Barrett CW, Stengel KR, Revetta FL, Choksi YA, Coburn LA, Lintel MK, McDonough EM, Washington MK, Wilson KT, Prokhortchouk E, Chen X, Hiebert SW, Reynolds AB, Williams CS., Free PMC Article

    12/21/2019
    The here presented transcriptomic subgroup-based approach unified the gene expression profiles of RUNX1-CBFA2T3 and RUNX1-RUNX1T1 acute myeloid leukemia.

    RNA-sequencing analysis of core binding factor AML identifies recurrent ZBTB7A mutations and defines RUNX1-CBFA2T3 fusion signature.
    Lavallée VP, Lemieux S, Boucher G, Gendron P, Boivin I, Armstrong RN, Sauvageau G, Hébert J.

    01/27/2018
    Analysis of chromatin immunoprecipitation sequencing data sets for MTGR1 and MTG16 targets indicated that MTGR1 can regulate Wnt and Notch signaling.

    Myeloid translocation genes differentially regulate colorectal cancer programs.
    Parang B, Bradley AM, Mittal MK, Short SP, Thompson JJ, Barrett CW, Naik RD, Bilotta AJ, Washington MK, Revetta FL, Smith JJ, Chen X, Wilson KT, Hiebert SW, Williams CS., Free PMC Article

    09/16/2017
    CBFA2T3 expression is regulated by Med19 in breast cancer cells.

    Med19 promotes breast cancer cell proliferation by regulating CBFA2T3/HEB expression.
    Zhang X, Fan Y, Liu B, Qi X, Guo Z, Li L.

    07/15/2017
    specific interference with ETO2-GLIS2 oligomerization reverses the transcriptional activation at enhancers and promotes megakaryocytic differentiation, providing a relevant interface to target in this poor-prognosis pediatric leukemia.

    ETO2-GLIS2 Hijacks Transcriptional Complexes to Drive Cellular Identity and Self-Renewal in Pediatric Acute Megakaryoblastic Leukemia.
    Thirant C, Ignacimouttou C, Lopez CK, Diop M, Le Mouël L, Thiollier C, Siret A, Dessen P, Aid Z, Rivière J, Rameau P, Lefebvre C, Khaled M, Leverger G, Ballerini P, Petit A, Raslova H, Carmichael CL, Kile BT, Soler E, Crispino JD, Wichmann C, Pflumio F, Schwaller J, Vainchenker W, Lobry C, Droin N, Bernard OA, Malinge S, Mercher T.

    06/24/2017
    Clinical courses of pediatric patients with AMKL harboring the CBFA2T3-GLIS2 fusion gene are poor due to resistance to chemotherapies and SCT. New treatment strategies are necessary.

    Clinical Courses of Two Pediatric Patients with Acute Megakaryoblastic Leukemia Harboring the CBFA2T3-GLIS2 Fusion Gene.
    Ishibashi M, Yokosuka T, Yanagimachi MD, Iwasaki F, Tsujimoto SI, Sasaki K, Takeuchi M, Tanoshima R, Kato H, Kajiwara R, Tanaka F, Goto H, Yokota S., Free PMC Article

    03/18/2017
    ETO2 and IRF2BP2 interacting with the NCOR1/SMRT co-repressor complex, suppresses the expression of erythroid genes until erythroid differentiation.

    Control of developmentally primed erythroid genes by combinatorial co-repressor actions.
    Stadhouders R, Cico A, Stephen T, Thongjuea S, Kolovos P, Baymaz HI, Yu X, Demmers J, Bezstarosti K, Maas A, Barroca V, Kockx C, Ozgur Z, van Ijcken W, Arcangeli ML, Andrieu-Soler C, Lenhard B, Grosveld F, Soler E., Free PMC Article

    07/2/2016
    MTG16 co-repressor promotes degradation of HIF1alpha in lymphoblasts.

    Myeloid translocation gene-16 co-repressor promotes degradation of hypoxia-inducible factor 1.
    Kumar P, Gullberg U, Olsson I, Ajore R., Free PMC Article

    04/16/2016
    study defined eight additional recurrently mutated genes in SMZL; these genes are CREBBP, CBFA2T3, AMOTL1, FAT4, FBXO11, PLA2G4D, TRRAP and USH2A.

    Whole exome sequencing identifies novel recurrently mutated genes in patients with splenic marginal zone lymphoma.
    Parry M, Rose-Zerilli MJ, Gibson J, Ennis S, Walewska R, Forster J, Parker H, Davis Z, Gardiner A, Collins A, Oscier DG, Strefford JC., Free PMC Article

    04/4/2015
    Findings are unprecedented and indicate that the DHH-RHEBL1 fusion transcript is a novel recurrent feature in the changing landscape of CBFA2T3-GLIS2-positive childhood AML.

    DHH-RHEBL1 fusion transcript: a novel recurrent feature in the new landscape of pediatric CBFA2T3-GLIS2-positive acute myeloid leukemia.
    Masetti R, Togni M, Astolfi A, Pigazzi M, Manara E, Indio V, Rizzari C, Rutella S, Basso G, Pession A, Locatelli F., Free PMC Article

    07/26/2014
    Expression of MTG16 reduced glycolytic metabolism while mitochondrial respiration and formation of reactive oxygen species increased.

    The transcriptional co-repressor myeloid translocation gene 16 inhibits glycolysis and stimulates mitochondrial respiration.
    Kumar P, Sharoyko VV, Spégel P, Gullberg U, Mulder H, Olsson I, Ajore R., Free PMC Article

    02/8/2014
    presence of MTG16 in this complex, and its contributions to transcriptional repression both required Kaiso binding to its binding site on DNA, establishing MTG16-Kaiso binding as functionally relevant in Kaiso-dependent transcriptional repression

    Kaiso directs the transcriptional corepressor MTG16 to the Kaiso binding site in target promoters.
    Barrett CW, Smith JJ, Lu LC, Markham N, Stengel KR, Short SP, Zhang B, Hunt AA, Fingleton BM, Carnahan RH, Engel ME, Chen X, Beauchamp RD, Wilson KT, Hiebert SW, Reynolds AB, Williams CS., Free PMC Article

    06/15/2013
    Data indicate taht ETO2/CBFA2T3 regulates GATA-1 target genes critical for erythroid differentiation, and the decrease of ETO2 levels during erythroid differentiation would contribute to the activation of these targets.

    Role of transcriptional corepressor ETO2 in erythroid cells.
    Fujiwara T, Alqadi YW, Okitsu Y, Fukuhara N, Onishi Y, Ishizawa K, Harigae H.

    05/25/2013
    Identification of a cryptic chromosome 16 inversion (inv(16)(p13.3q24.3)) in 27% of pediatric acute megakaryoblastic leukemia cases, which encodes a CBFA2T3-GLIS2 fusion protein.

    An Inv(16)(p13.3q24.3)-encoded CBFA2T3-GLIS2 fusion protein defines an aggressive subtype of pediatric acute megakaryoblastic leukemia.
    Gruber TA, Larson Gedman A, Zhang J, Koss CS, Marada S, Ta HQ, Chen SC, Su X, Ogden SK, Dang J, Wu G, Gupta V, Andersson AK, Pounds S, Shi L, Easton J, Barbato MI, Mulder HL, Manne J, Wang J, Rusch M, Ranade S, Ganti R, Parker M, Ma J, Radtke I, Ding L, Cazzaniga G, Biondi A, Kornblau SM, Ravandi F, Kantarjian H, Nimer SD, Döhner K, Döhner H, Ley TJ, Ballerini P, Shurtleff S, Tomizawa D, Adachi S, Hayashi Y, Tawa A, Shih LY, Liang DC, Rubnitz JE, Pui CH, Mardis ER, Wilson RK, Downing JR., Free PMC Article

    01/26/2013
    An evolutionary conserved GATA binding site is critical in transcriptional regulation of the MTG16 promoter.

    The leukemia associated nuclear corepressor ETO homologue genes MTG16 and MTGR1 are regulated differently in hematopoietic cells.
    Ajore R, Kumar P, Dhanda RS, Gullberg U, Olsson I., Free PMC Article

    09/22/2012
    Our data indicate that the CBFA2T3/ACSF3 locus is a novel recurrent oncogenic target of immunoglobulin heavy chain translocations, which might contribute to the pathogenesis of pediatric GC-derived B-cell lymphoma.

    The CBFA2T3/ACSF3 locus is recurrently involved in IGH chromosomal translocation t(14;16)(q32;q24) in pediatric B-cell lymphoma with germinal center phenotype.
    Salaverria I, Akasaka T, Gesk S, Szczepanowski M, Burkhardt B, Harder L, Damm-Welk C, Oschlies I, Klapper W, Dyer MJ, Siebert R.

    06/16/2012
    Alternative NLI complexes mediate gamma-globin transcription or silencing through long-range locus control region interactions involving an intergenic site of noncoding RNA transcription and that ETO2 is critical to this process.

    Distinct Ldb1/NLI complexes orchestrate γ-globin repression and reactivation through ETO2 in human adult erythroid cells.
    Kiefer CM, Lee J, Hou C, Dale RK, Lee YT, Meier ER, Miller JL, Dean A., Free PMC Article

    01/28/2012
    Study provides the first evidence that MTG16b is a dual AKAP capable of binding plexins.

    Myeloid translocation gene 16b is a dual A-kinase anchoring protein that interacts selectively with plexins in a phospho-regulated manner.
    Fiedler SE, Schillace RV, Daniels CJ, Andrews SF, Carr DW.

    03/22/2010
    Data show that ZNF651 is a ZNF652 paralogue that shares a common DNA binding sequence with ZNF652 and represses target gene expression through the formation of a CBFA2T3-ZNF651 corepressor complex.

    CBFA2T3-ZNF651, like CBFA2T3-ZNF652, functions as a transcriptional corepressor complex.
    Kumar R, Cheney KM, Neilsen PM, Schulz RB, Callen DF.

    03/22/2010
    ETO family member-mediated oligomerization and repression can be distinct events and that interaction between ETO family members and hSIN3B or N-CoR may not necessarily strengthen transcriptional repression.

    Transcriptional repression by leukaemia-associated ETO family members can be independent of oligomerization and coexpressed hSIN3B and N-CoR.
    Olsson A, Olsson I, Dhanda RS.

    01/21/2010
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