IKZF1

IKZF1

Target · Transcription factor

IKZF1 IKAROS family zinc finger 1 · DNA-binding protein Ikaros

IKZF1 (IKAROS family zinc finger 1), also known as Ikaros, hIk-1, LyF-1, PPP1R92 and ZNFN1A1, is a C2H2-type zinc-finger transcription regulator of haematopoietic cell differentiation. It has a central role in the development of lymphocytes, including B and T cells. Browse Assay Genie's current range of IKZF1 research reagents below, or read on for the biology behind the target.

Nuclear and cytoplasmicLymphocyte differentiation regulatorIsoform-specific function
519 aa
Protein length
~58 kDa
Molecular weight
7p12.2
Gene locus
Zinc finger
Protein family
Target information

What is IKZF1 (IKAROS family zinc finger 1)?

IKZF1 is a transcription regulator that controls haematopoietic cell differentiation and contributes to the development of lymphocytes, B cells and T cells. It binds gamma-satellite DNA, binds and activates the delta-A enhancer element of the CD3-delta gene, and represses the terminal deoxynucleotidyltransferase (TDT) gene during thymocyte differentiation. It regulates transcription through both HDAC-dependent and HDAC-independent complexes. In adult erythrocytes it targets the NuRD and BAF (SWI/SNF) chromatin-remodelling complexes, together as the PYR complex, to the beta-globin locus, and it increases normal apoptosis in adult erythroid cells. It also confers early temporal competence to retinal progenitor cells.

The IKZF1 gene encodes a member of the Ikaros family of zinc-finger DNA-binding proteins associated with chromatin remodelling. Expression of the IKZF1 protein is restricted to the foetal and adult haemo-lymphopoietic system. Alternative splicing gives several isoforms. Most share a C-terminal domain with two zinc fingers that mediate homo- and hetero-dimerisation and interactions with other proteins. The isoforms differ in the number of N-terminal zinc fingers that bind DNA and in the presence of a nuclear localisation signal. Only a few contain the three or more N-terminal fingers needed for high-affinity binding to a core DNA element in target promoters. Isoforms that cannot bind DNA are largely cytoplasmic and are thought to act as dominant-negative factors, so IKZF1 function is isoform-specific.

Overexpression of some dominant-negative isoforms has been associated with B-cell malignancies such as acute lymphoblastic leukaemia. IKZF1 is recognised by CRBN and targeted for degradation by the DCX (DDB1-CUL4-X-box) E3 protein ligase complex. HGNC places the gene in the C2H2-type zinc finger group and among protein phosphatase 1 regulatory subunits, reflected in the alias PPP1R92.

Lymphocyte development

IKZF1 regulates differentiation of haematopoietic cells and contributes to the development of B and T lymphocytes.

Chromatin remodelling

IKZF1 acts through HDAC-dependent and HDAC-independent complexes and directs the NuRD and BAF complexes to the beta-globin locus.

B-cell leukaemia research

Overexpression of dominant-negative IKZF1 isoforms has been associated with B-cell malignancies such as acute lymphoblastic leukaemia.

Targeted protein degradation

IKZF1 is recognised by CRBN and targeted for degradation by the DCX E3 ubiquitin ligase complex.

Background

IKZF1 frequently asked questions

What does IKZF1 do?

IKZF1 is a transcription regulator of haematopoietic cell differentiation and lymphocyte development. It binds DNA elements such as the CD3-delta enhancer, represses the TDT gene in thymocytes, and works with chromatin-remodelling complexes including NuRD and BAF to regulate target loci.

What other names and isoforms does IKZF1 have?

IKZF1 is also called Ikaros, hIk-1, LyF-1, PPP1R92 and ZNFN1A1, and was formerly named zinc finger protein, subfamily 1A, 1 (Ikaros). Alternative splicing yields isoforms that differ in N-terminal zinc fingers and nuclear localisation signal, so some bind DNA and others do not.

Is IKZF1 conserved and linked to disease?

IKZF1 has a mouse ortholog, Ikzf1. Overexpression of some dominant-negative isoforms has been associated with B-cell malignancies such as acute lymphoblastic leukaemia. Check the datasheet of any product for its validated reactivity.

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Related targets

Other Ikaros family zinc-finger transcription factors with products at Assay Genie.

Gene and protein annotations from HGNC, NCBI Gene and UniProt Q13422; product specifications from Assay Genie datasheets.

IKZF1 Product listing