IKZF2 (IKAROS family zinc finger 2)

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IKZF2 (IKAROS family zinc finger 2)

Identity

Other names Helios

ZNFN1A2

Mouse Ikzf2

HGNC IKZF2

Location 2q34

Note Belongs to the Ikaros family of zinc finger containing DNA binding proteins.

DNA/RNA

Description 143kb encoding 7 exons.

Transcription 12kb transcript, alternatively spliced to give two predominant isoforms.

Protein

Helios with the zinc finger domain shown as blocks and the exon junctions in black lines.

Note 526 amino acids. Mouse protein migrates at 70kD.

Description Largest isoform contains four N-terminal zinc fingers used for DNA binding and two C terminal zinc fingers for homodimerization and heterodimerization with other Ikaros family members.

Expression Largely restricted to the thymus. In mice, besides thymocytes and T cells, low levels are found in proB cells and bone marrow multipotent progenitors.

Localisation Nuclear. Often localizes to the pericentric heterochromatin as punctate spots in cycling cells, with diffuse nuclear localization in non-cycling cells.

Function Helios is thought to be important for T cell development and may function as a repressor of transcription. However, Helios mutant mice have not been reported. In mice, Helios can recruit the NuRD chromatin remodeling complex to the pericentric heterochromatin.

Homology High level of identity to Ikaros over the zinc finger domains.

Implicated in

Entity Leukemia

Note Shorter isoforms of Helios are expressed in certain human leukemias. They may function as dominant negative inhibitors of full-length proteins due to the lack of DNA binding domain. However, dominant negative functions have not been demonstrated experimentally.

Disease T cell acute lymphoblastic leukemia.

Entity Lymphoma

Note Overexpression of Helios in B cells of mice promotes lymphomagenesis. Overexpression of a DNA binding mutant in hematopoietic progenitors leads to aggressive and transplantable T cell lymphomas in 60% of the mice.

External links

	Nomenclature
HGNC	IKZF2 13177
Entrez_Gene	IKZF2 22807 IKAROS family zinc finger 2 (Helios)
	Cards
Atlas	IKZF2ID42885ch2q34
GeneCards	IKZF2
Ensembl	IKZF2 [Search_View] ENSG00000030419 [Gene_View]
Genatlas	IKZF2
GeneLynx	IKZF2
eGenome	IKZF2
euGene	22807
	Genomic and cartography
GoldenPath	IKZF2 - 2q34 chr2:213572656-213724578 - 2q13.1 [Description] (hg18-Mar_2006)
Ensembl	IKZF2 - 2q13.1 [CytoView]
NCBI	Mapview
OMIM	Disease map [OMIM]
HomoloGene	IKZF2
	Gene and transcription
Genbank	AF130863 [ ENTREZ ]
Genbank	AI458439 [ ENTREZ ]
Genbank	AY008294 [ ENTREZ ]
Genbank	AY587062 [ ENTREZ ]
Genbank	AY587063 [ ENTREZ ]
RefSeq	NM_001079526 [ SRS ] NM_001079526 [ ENTREZ ]
RefSeq	NM_016260 [ SRS ] NM_016260 [ ENTREZ ]
RefSeq	AC_000045 [ SRS ] AC_000045 [ ENTREZ ]
RefSeq	AC_000134 [ SRS ] AC_000134 [ ENTREZ ]
RefSeq	NC_000002 [ SRS ] NC_000002 [ ENTREZ ]
RefSeq	NT_005403 [ SRS ] NT_005403 [ ENTREZ ]
RefSeq	NW_001838863 [ SRS ] NW_001838863 [ ENTREZ ]
RefSeq	NW_921618 [ SRS ] NW_921618 [ ENTREZ ]
AceView	IKZF2 AceView - NCBI
Unigene	Hs.604950 [ SRS ] Hs.604950 [ NCBI ] HS604950 [ spliceNest ]
Fast-db	14877 (alternative variants)
	Protein : pattern, domain, 3D structure
SwissProt	Q53QP1 [ SRS] Q53QP1 [ EXPASY ] Q53QP1 [ INTERPRO ] Q53QP1 [ UNIPROT ]
Prosite	PS00028 ZINC_FINGER_C2H2_1 [ SRS ] PS00028 ZINC_FINGER_C2H2_1 [ Expasy ]
Prosite	PS50157 ZINC_FINGER_C2H2_2 [ SRS ] PS50157 ZINC_FINGER_C2H2_2 [ Expasy ]
Interpro	IPR007087 Znf_C2H2 [ SRS ] IPR007087 Znf_C2H2 [ EBI ]
Interpro	IPR015880 Znf_C2H2-like [ SRS ] IPR015880 Znf_C2H2-like [ EBI ]
CluSTr	Q53QP1
Pfam	PF00096 zf-C2H2 [ SRS ] PF00096 zf-C2H2 [ Sanger ] pfam00096 [ NCBI-CDD ]
Smart	SM00355 ZnF_C2H2 [EMBL]
Blocks	Q53QP1
HPRD	05872
	Protein Interaction databases
DIP	Q53QP1
IntAct	Q53QP1
	Polymorphism : SNP, mutations, diseases
OMIM	606234 [ map ]
GENECLINICS	606234
SNP	IKZF2 [dbSNP-NCBI]
SNP	NM_001079526 [SNP-NCI]
SNP	NM_016260 [SNP-NCI]
SNP	IKZF2 [GeneSNPs - Utah] IKZF2] [HGBASE - SRS]
HAPMAP	IKZF2 [HAPMAP]
COSMIC	IKZF2 [Somatic mutation (COSMIC-CGP-Sanger)]
HGMD	IKZF2
	General knowledge
Family Browser	IKZF2 [UCSC Family Browser]
SOURCE	NM_001079526
SOURCE	NM_016260
SMD	Hs.604950
SAGE	Hs.604950
GO	molecular_function [Amigo] molecular_function
GO	nucleic acid binding [Amigo] nucleic acid binding
GO	DNA binding [Amigo] DNA binding
GO	intracellular [Amigo] intracellular
GO	nucleus [Amigo] nucleus
GO	transcription [Amigo] transcription
GO	regulation of transcription, DNA-dependent [Amigo] regulation of transcription, DNA-dependent
GO	biological_process [Amigo] biological_process
GO	zinc ion binding [Amigo] zinc ion binding
GO	metal ion binding [Amigo] metal ion binding
PubGene	IKZF2
TreeFam	IKZF2
CTD	22807 [Comparative ToxicoGenomics Database]
	Other databases
	Probes
Probe	IKZF2 Related clones (RZPD - Berlin)
	PubMed
PubMed	16 Pubmed reference(s) in LocusLink

Bibliography

Helios, a T cell-restricted Ikaros family member that quantitatively associates with Ikaros at centromeric heterochromatin.

Hahm K, Cobb BS, McCarty AS, Brown KE, Klug CA, Lee R, Akashi K, Weissman IL, Fisher AG, Smale ST

Genes & development. 1998 ; 12 (6) : 782-796.

PMID 9512513

Helios, a novel dimerization partner of Ikaros expressed in the earliest hematopoietic progenitors.

Kelley CM, Ikeda T, Koipally J, Avitahl N, Wu L, Georgopoulos K, Morgan BA

Current biology : CB. 1998 ; 8 (9) : 508-515.

PMID 9560339

Human Helios, an Ikaros-related zinc finger DNA binding protein: cDNA cloning and tissue expression pattern.

Hosokawa Y, Maeda Y, Seto M

Immunogenetics. 1999 ; 50 (1-2) : 106-108.

PMID 10541817

Overexpression of novel short isoforms of Helios in a patient with T-cell acute lymphoblastic leukemia.

Nakase K, Ishimaru F, Fujii K, Tabayashi T, Kozuka T, Sezaki N, Matsuo Y, Harada M

Experimental hematology. 2002 ; 30 (4) : 313-317.

PMID 11937265

Transgenic expression of Helios in B lineage cells alters B cell properties and promotes lymphomagenesis.

Dovat S, Montecino-Rodriguez E, Schuman V, Teitell MA, Dorshkind K, Smale ST

Journal of immunology (Baltimore, Md. : 1950). 2005 ; 175 (6) : 3508-3515.

PMID 16148093

Expression of a non-DNA-binding isoform of Helios induces T-cell lymphoma in mice.

Zhang Z, Swindle CS, Bates JT, Ko R, Cotta CV, Klug CA

Blood. 2007 ; 109 (5) : 2190-2197.

PMID 17110463

REVIEW articles automatic search in PubMed

Last year publications automatic search in PubMed

Search in all EBI NCBI

Contributor(s)

Written 04-2007 Rupa Sridharan, Stephen Smale

Room 6730 MacDonald Research Laboratories, Howard Hughes Medical Institute, 675 Charles Young Drive South, University of California, Los Angeles, CA 90095-1662, USA.

Citation

This paper should be referenced as such :
Sridharan R, Smale S . IKZF2 (IKAROS family zinc finger 2). Atlas Genet Cytogenet Oncol Haematol. April 2007 .
URL : http://AtlasGeneticsOncology.org/Genes/IKZF2ID42885ch2q34.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Mon Aug 11 21:14:32 2008

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For comments and suggestions or contributions, please contact us
j.l.huret@chu-poitiers.fr.

Other names	Helios
	ZNFN1A2
	Mouse Ikzf2
HGNC	IKZF2
Location	2q34

Description	143kb encoding 7 exons.
Transcription	12kb transcript, alternatively spliced to give two predominant isoforms.

Note	526 amino acids. Mouse protein migrates at 70kD.
Description	Largest isoform contains four N-terminal zinc fingers used for DNA binding and two C terminal zinc fingers for homodimerization and heterodimerization with other Ikaros family members.
Expression	Largely restricted to the thymus. In mice, besides thymocytes and T cells, low levels are found in proB cells and bone marrow multipotent progenitors.
Localisation	Nuclear. Often localizes to the pericentric heterochromatin as punctate spots in cycling cells, with diffuse nuclear localization in non-cycling cells.
Function	Helios is thought to be important for T cell development and may function as a repressor of transcription. However, Helios mutant mice have not been reported. In mice, Helios can recruit the NuRD chromatin remodeling complex to the pericentric heterochromatin.
Homology	High level of identity to Ikaros over the zinc finger domains.

Entity	Leukemia
Note	Shorter isoforms of Helios are expressed in certain human leukemias. They may function as dominant negative inhibitors of full-length proteins due to the lack of DNA binding domain. However, dominant negative functions have not been demonstrated experimentally.
Disease	T cell acute lymphoblastic leukemia.

Entity	Lymphoma
Note	Overexpression of Helios in B cells of mice promotes lymphomagenesis. Overexpression of a DNA binding mutant in hematopoietic progenitors leads to aggressive and transplantable T cell lymphomas in 60% of the mice.

REVIEW articles	automatic search in PubMed
Last year publications	automatic search in PubMed

Written	04-2007	Rupa Sridharan, Stephen Smale
		Room 6730 MacDonald Research Laboratories, Howard Hughes Medical Institute, 675 Charles Young Drive South, University of California, Los Angeles, CA 90095-1662, USA.