Download Gene Section IKZF1 (Ikaros family zinc finger 1) in Oncology and Haematology

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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