Ikaros and acute leukemia

摘要

The role of Ikaros in the pathogenesis of acute lymphoblastic leukemia (ALL) has been an enigma for many years. Ikaros is a member of a family of zinc finger transcription factors that also includes Helios, Aiolos, Eos and Pegasus. Ikaros is the best characterised of these, and has key roles in lymphoid development and tumour suppression. Ikaros is encoded by the IKZF1 gene (also known as ZNFN1A1) at 7pl2 which comprises 8 exons, 7 of which are coding. Exons 3-5 encode 4 N-terminal zinc fingers (ZnFs) required for DNA binding, and exon 7 encodes two C-terminal ZnFs that mediate homo-and heterodimerization with Ikaros family members , Alternative splicing can generate multiple Ikaros isoforms that lack variable numbers of internal exons (Figure 1)