CBFA2T2 explained

Protein CBFA2T2 is a protein that in humans is encoded by the CBFA2T2 gene.^{[1] [2]}

Function

In acute myeloid leukemia, especially in the M2 subtype, the t(8;21)(q22;q22) translocation is one of the most frequent karyotypic abnormalities. The translocation produces a chimeric gene made up of the 5'-region of the RUNX1 (AML1) gene fused to the 3'-region of the CBFA2T1 (MTG8) gene. The chimeric protein is thought to associate with the nuclear corepressor/histone deacetylase complex to block hematopoietic differentiation. The protein encoded by this gene binds to the AML1-MTG8 complex and may be important in promoting leukemogenesis. Several transcript variants are thought to exist for this gene, but the full-length natures of only three have been described.^[2]

Interactions

CBFA2T2 has been shown to interact with RUNX1T1.^{[3] [4] [5]}

Further reading

• Kitabayashi I, Ida K, Morohoshi F, Yokoyama A, Mitsuhashi N, Shimizu K, Nomura N, Hayashi Y, Ohki M . 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 . 1998 . 9447981 . 108796 . 10.1128/MCB.18.2.846.
• Fracchiolla NS, Colombo G, Finelli P, Maiolo AT, Neri A . EHT, a new member of the MTG8/ETO gene family, maps on 20q11 region and is deleted in acute myeloid leukemias. . Blood . 92 . 9 . 3481–4 . 1998 . 9787195 . 10.1182/blood.V92.9.3481.
• Morohoshi F, Mitani S, Mitsuhashi N, Kitabayashi I, Takahashi E, Suzuki M, Munakata N, Ohki M . Structure and expression pattern of a human MTG8/ETO family gene, MTGR1. . Gene . 241 . 2 . 287–95 . 2000 . 10675041 . 10.1016/S0378-1119(99)00481-3 .
• Hoogeveen AT, Rossetti S, Stoyanova V, Schonkeren J, Fenaroli A, Schiaffonati L, van Unen L, Sacchi N . The transcriptional corepressor MTG16a contains a novel nucleolar targeting sequence deranged in t (16; 21)-positive myeloid malignancies. . Oncogene . 21 . 43 . 6703–12 . 2002 . 12242670 . 10.1038/sj.onc.1205882 . free .
• Lindberg SR, Olsson A, Persson AM, Olsson I . Interactions between the leukaemia-associated ETO homologues of nuclear repressor proteins. . Eur. J. Haematol. . 71 . 6 . 439–47 . 2004 . 14703694 . 10.1046/j.0902-4441.2003.00166.x . 23106882 .
• Brandenberger R, Wei H, Zhang S, Lei S, Murage J, Fisk GJ, Li Y, Xu C, Fang R, Guegler K, Rao MS, Mandalam R, Lebkowski J, Stanton LW . Transcriptome characterization elucidates signaling networks that control human ES cell growth and differentiation. . Nat. Biotechnol. . 22 . 6 . 707–16 . 2005 . 15146197 . 10.1038/nbt971 . 27764390 .
• Beausoleil SA, Jedrychowski M, Schwartz D, Elias JE, Villén J, Li J, Cohn MA, Cantley LC, Gygi SP . Large-scale characterization of HeLa cell nuclear phosphoproteins. . Proc. Natl. Acad. Sci. U.S.A. . 101 . 33 . 12130–5 . 2004 . 15302935 . 514446 . 10.1073/pnas.0404720101 . 2004PNAS..10112130B . free .
• Zhang J, Kalkum M, Yamamura S, Chait BT, Roeder RG . E protein silencing by the leukemogenic AML1-ETO fusion protein. . Science . 305 . 5688 . 1286–9 . 2004 . 15333839 . 10.1126/science.1097937 . 2004Sci...305.1286Z . 850967 .
• Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M . Towards a proteome-scale map of the human protein–protein interaction network. . Nature . 437 . 7062 . 1173–8 . 2005 . 16189514 . 10.1038/nature04209 . 2005Natur.437.1173R . 4427026 .
• Lim J, Hao T, Shaw C, Patel AJ, Szabó G, Rual JF, Fisk CJ, Li N, Smolyar A, Hill DE, Barabási AL, Vidal M, Zoghbi HY . A protein–protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration. . Cell . 125 . 4 . 801–14 . 2006 . 16713569 . 10.1016/j.cell.2006.03.032 . 13709685 . free .
• Kumar R, Manning J, Spendlove HE, Kremmidiotis G, McKirdy R, Lee J, Millband DN, Cheney KM, Stampfer MR, Dwivedi PP, Morris HA, Callen DF . ZNF652, a novel zinc finger protein, interacts with the putative breast tumor suppressor CBFA2T3 to repress transcription. . Mol. Cancer Res. . 4 . 9 . 655–65 . 2006 . 16966434 . 10.1158/1541-7786.MCR-05-0249 . free .

Notes and References

1. Calabi F, Cilli V . CBFA2T1, a gene rearranged in human leukemia, is a member of a multigene family . Genomics . 52 . 3 . 332–41 . December 1998 . 9790752 . 10.1006/geno.1998.5429 .
2. Web site: Entrez Gene: CBFA2T2 core-binding factor, runt domain, alpha subunit 2; translocated to, 2.
3. Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M . Towards a proteome-scale map of the human protein–protein interaction network. Huda Zoghbi . . 437 . 7062 . 1173–8 . October 2005 . 16189514 . 10.1038/nature04209 . 2005Natur.437.1173R. 4427026.
4. Lindberg SR, Olsson A, Persson AM, Olsson I . Interactions between the leukaemia-associated ETO homologues of nuclear repressor proteins . Eur. J. Haematol. . 71 . 6 . 439–47 . December 2003 . 14703694 . 10.1046/j.0902-4441.2003.00166.x . 23106882 .
5. Hoogeveen AT, Rossetti S, Stoyanova V, Schonkeren J, Fenaroli A, Schiaffonati L, van Unen L, Sacchi N . The transcriptional corepressor MTG16a contains a novel nucleolar targeting sequence deranged in t (16; 21)-positive myeloid malignancies . Oncogene . 21 . 43 . 6703–12 . September 2002 . 12242670 . 10.1038/sj.onc.1205882 . free .