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Core-binding factor, runt domain, alpha subunit 2; translocated to, 3

PDB rendering based on 2h7b.
Available structures
Symbols CBFA2T3; ETO2; MTG16; MTGR2; ZMYND4
External IDs OMIM603870 MGI1338013 HomoloGene74543 GeneCards: CBFA2T3 Gene
RNA expression pattern
PBB GE CBFA2T3 208056 s at tn.png
More reference expression data
Species Human Mouse
Entrez 863 12398
Ensembl ENSG00000129993 ENSMUSG00000006362
UniProt n/a n/a
RefSeq (mRNA) NM_005187 NM_009824
RefSeq (protein) NP_005178 NP_033954
Location (UCSC) Chr 16:
87.47 - 87.57 Mb
Chr 8:
125.52 - 125.59 Mb
PubMed search [1] [2]

Protein CBFA2T3 is a protein that in humans is encoded by the CBFA2T3 gene.[1][2]

The t(16;21)(q24;q22) translocation is a rare but recurrent chromosomal abnormality associated with therapy-related myeloid malignancies. The translocation produces a chimeric gene made up of the 5'-region of the AML1 gene fused to the 3'-region of this gene. In addition, this gene is a putative breast tumor suppressor. Two transcript variants encoding different isoforms have been found for this gene, and a brefeldin A-sensitive association of RII-alpha protein with one of the isoforms has been demonstrated in the Golgi apparatus.[2]



CBFA2T3 has been shown to interact with HDAC1,[3][4] HDAC3,[3][4] TCF3,[5] LDB1,[5] TAL1,[5] RUNX1T1[6][3] and PRKAR2A.[7]


  1. ^ Calabi F, Cilli V (Dec 1998). "CBFA2T1, a gene rearranged in human leukemia, is a member of a multigene family". Genomics 52 (3): 332–41. doi:10.1006/geno.1998.5429. PMID 9790752.  
  2. ^ a b "Entrez Gene: CBFA2T3 core-binding factor, runt domain, alpha subunit 2; translocated to, 3".  
  3. ^ a b c Hoogeveen, André T; Rossetti Stefano, Stoyanova Violeta, Schonkeren Joris, Fenaroli Angelia, Schiaffonati Luisa, van Unen Leontine, Sacchi Nicoletta (Sep. 2002). "The transcriptional corepressor MTG16a contains a novel nucleolar targeting sequence deranged in t (16; 21)-positive myeloid malignancies". Oncogene (England) 21 (43): 6703–12. doi:10.1038/sj.onc.1205882. ISSN 0950-9232. PMID 12242670.  
  4. ^ a b Amann, J M; Nip J, Strom D K, Lutterbach B, Harada H, Lenny N, Downing J R, Meyers S, Hiebert S W (Oct. 2001). "ETO, a target of t(8;21) in acute leukemia, makes distinct contacts with multiple histone deacetylases and binds mSin3A through its oligomerization domain". Mol. Cell. Biol. (United States) 21 (19): 6470–83. ISSN 0270-7306. PMID 11533236.  
  5. ^ a b c Goardon, Nicolas; Lambert Julie A, Rodriguez Patrick, Nissaire Philippe, Herblot Sabine, Thibault Pierre, Dumenil Dominique, Strouboulis John, Romeo Paul-Henri, Hoang Trang (Jan. 2006). "ETO2 coordinates cellular proliferation and differentiation during erythropoiesis". EMBO J. (England) 25 (2): 357–66. doi:10.1038/sj.emboj.7600934. ISSN 0261-4189. PMID 16407974.  
  6. ^ Lindberg, Sofia Rondin; Olsson André, Persson Ann-Maj, Olsson Inge (Dec. 2003). "Interactions between the leukaemia-associated ETO homologues of nuclear repressor proteins". Eur. J. Haematol. (Denmark) 71 (6): 439–47. ISSN 0902-4441. PMID 14703694.  
  7. ^ Schillace, Robynn V; Andrews Sarah F, Liberty Greg A, Davey Michael P, Carr Daniel W (Feb. 2002). "Identification and characterization of myeloid translocation gene 16b as a novel a kinase anchoring protein in T lymphocytes". J. Immunol. (United States) 168 (4): 1590–9. ISSN 0022-1767. PMID 11823486.  

Further reading

  • Kitabayashi I, Ida K, Morohoshi F, et al. (1998). "The AML1-MTG8 leukemic fusion protein forms a complex with a novel member of the MTG8(ETO/CDR) family, MTGR1.". Mol. Cell. Biol. 18 (2): 846–58. PMID 9447981.  
  • Gamou T, Kitamura E, Hosoda F, et al. (1998). "The partner gene of AML1 in t(16;21) myeloid malignancies is a novel member of the MTG8(ETO) family.". Blood 91 (11): 4028–37. PMID 9596646.  
  • Schillace RV, Andrews SF, Liberty GA, et al. (2002). "Identification and characterization of myeloid translocation gene 16b as a novel a kinase anchoring protein in T lymphocytes.". J. Immunol. 168 (4): 1590–9. PMID 11823486.  
  • Kondoh K, Nakata Y, Furuta T, et al. (2003). "A pediatric case of secondary leukemia associated with t(16;21)(q24;q22) exhibiting the chimeric AML1-MTG16 gene.". Leuk. Lymphoma 43 (2): 415–20. doi:10.1080/10428190290006242. PMID 11999578.  
  • Kochetkova M, McKenzie OL, Bais AJ, et al. (2002). "CBFA2T3 (MTG16) is a putative breast tumor suppressor gene from the breast cancer loss of heterozygosity region at 16q24.3.". Cancer Res. 62 (16): 4599–604. PMID 12183414.  
  • Powell JA, Gardner AE, Bais AJ, et al. (2003). "Sequencing, transcript identification, and quantitative gene expression profiling in the breast cancer loss of heterozygosity region 16q24.3 reveal three potential tumor-suppressor genes.". Genomics 80 (3): 303–10. doi:10.1006/geno.2002.6828. PMID 12213200.  
  • Hoogeveen AT, Rossetti S, Stoyanova V, et al. (2002). "The transcriptional corepressor MTG16a contains a novel nucleolar targeting sequence deranged in t (16; 21)-positive myeloid malignancies.". Oncogene 21 (43): 6703–12. doi:10.1038/sj.onc.1205882. PMID 12242670.  
  • Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMID 12477932.  
  • Ota T, Suzuki Y, Nishikawa T, et al. (2004). "Complete sequencing and characterization of 21,243 full-length human cDNAs.". Nat. Genet. 36 (1): 40–5. doi:10.1038/ng1285. PMID 14702039.  
  • Lindberg SR, Olsson A, Persson AM, Olsson I (2004). "Interactions between the leukaemia-associated ETO homologues of nuclear repressor proteins.". Eur. J. Haematol. 71 (6): 439–47. doi:10.1046/j.0902-4441.2003.00166.x. PMID 14703694.  
  • Ibañez V, Sharma A, Buonamici S, et al. (2004). "AML1-ETO decreases ETO-2 (MTG16) interactions with nuclear receptor corepressor, an effect that impairs granulocyte differentiation.". Cancer Res. 64 (13): 4547–54. doi:10.1158/0008-5472.CAN-03-3689. PMID 15231665.  
  • Kumar R, Manning J, Spendlove HE, et al. (2006). "ZNF652, a novel zinc finger protein, interacts with the putative breast tumor suppressor CBFA2T3 to repress transcription.". Mol. Cancer Res. 4 (9): 655–65. doi:10.1158/1541-7786.MCR-05-0249. PMID 16966434.  

External links

This article incorporates text from the United States National Library of Medicine, which is in the public domain.



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