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Atlas of Genetics and Cytogenetics in Oncology and Haematology OPEN ACCESS JOURNAL AT INIST-CNRS Gene Section Mini Review IKZF1 (Ikaros family zinc finger 1) Jean-Loup Huret Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France (JLH) Published in Atlas Database: August 2000 Online updated version : http://AtlasGeneticsOncology.org/Genes/IkarosID258.html DOI: 10.4267/2042/37660 This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence. © 2000 Atlas of Genetics and Cytogenetics in Oncology and Haematology Identity Implicated in HGNC (Hugo): IKZF1 Other names: IK1; LYF1; ZNFN1A1 (zinc finger protein, subfamily 1A, 1) Location: 7p12 t(3;7)(q27;p12) diffuse large B-cell lymphoma (DLCL) --> BCL6 / Ikaros Note Only 2 cases to date. Hybrid/Mutated gene 5' Ikaros - 3' BCL6 fusion transcript; it is supposed that substitution of the promoter of BCL6 may be responsible for BCL6 deregulation. DNA/RNA Transcription 3629 bp mRNA; coding sequence: 1559 bp; 6 different splicings (--> 6 isoform proteins: ik1-ik6). References Protein Brown KE, Guest SS, Smale ST, Hahm K, Merkenschlager M, Fisher AG. Association of transcriptionally silent genes with Ikaros complexes at centromeric heterochromatin. Cell. 1997 Dec 12;91(6):845-54 Description 519 amino acids; 58 kDa; possesses 2 Zinc-finger (C2H2-type) domains: one with 4 ZnF, the second with 2 ZnF; DNA binding. Georgopoulos K, Winandy S, Avitahl N. The role of the Ikaros gene in lymphocyte development and homeostasis. Annu Rev Immunol. 1997;15:155-76 Expression Nichogiannopoulou A, Trevisan M, Friedrich C, Georgopoulos K. Ikaros in hemopoietic lineage determination and homeostasis. Semin Immunol. 1998 Apr;10(2):119-25 Specificity for hematopoietic organs in the foetus and in the adult as well. Nichogiannopoulou A, Trevisan M, Neben S, Friedrich C, Georgopoulos K. Defects in hemopoietic stem cell activity in Ikaros mutant mice. J Exp Med. 1999 Nov 1;190(9):1201-14 Localisation Nuclear. Winandy S, Wu L, Wang JH, Georgopoulos K. Pre-T cell receptor (TCR) and TCR-controlled checkpoints in T cell differentiation are set by Ikaros. J Exp Med. 1999 Oct 18;190(8):1039-48 Function Transcription regulator; can repress transcription through the recruitment of histone deacetylase complexes; role in conjunction with Aiolos; hemopoietic-specific zinc finger protein regulator of B and T-cell differentiation. Atlas Genet Cytogenet Oncol Haematol. 2000; 4(4) Yoshida S, Kaneita Y, Aoki Y, Seto M, Mori S, Moriyama M. Identification of heterologous translocation partner genes fused to the BCL6 gene in diffuse large B-cell lymphomas: 5'-RACE and LA - PCR analyses of biopsy samples. Oncogene. 1999 Dec 23;18(56):7994-9 179 IKZF1 (Ikaros family zinc finger 1) Huret JL Hosokawa Y, Maeda Y, Ichinohasama R, Miura I, Taniwaki M, Seto M. The Ikaros gene, a central regulator of lymphoid differentiation, fuses to the BCL6 gene as a result of t(3;7)(q27;p12) translocation in a patient with diffuse large Bcell lymphoma. Blood. 2000 Apr 15;95(8):2719-21 This article should be referenced as such: Huret JL. IKZF1 (Ikaros family zinc finger 1). Atlas Genet Cytogenet Oncol Haematol. 2000; 4(4):179-180. Koipally J, Georgopoulos K. Ikaros interactions with CtBP reveal a repression mechanism that is independent of histone deacetylase activity. J Biol Chem. 2000 Jun 30;275(26):19594602 Atlas Genet Cytogenet Oncol Haematol. 2000; 4(4) 180