Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2012 Jan 1;17(3):1120-39.
doi: 10.2741/3977.

RUNX1 and RUNX1-ETO: roles in hematopoiesis and leukemogenesis

Kentson Lam  1 , Dong-Er Zhang
Affiliations
Review

RUNX1 and RUNX1-ETO: roles in hematopoiesis and leukemogenesis

Kentson Lam et al. Front Biosci (Landmark Ed). .

Abstract

RUNX1 is a transcription factor that regulates critical processes in many aspects of hematopoiesis. RUNX1 is also integral in defining the definitive hematopoietic stem cell. In addition, many hematological diseases like myelodysplastic syndrome and myeloproliferative neoplasms have been associated with mutations in RUNX1. Located on chromosomal 21, the RUNX1 gene is involved in many forms of chromosomal translocations in leukemia. t(8;21) is one of the most common chromosomal translocations found in acute myeloid leukemia (AML), where it results in a fusion protein between RUNX1 and ETO. The RUNX1-ETO fusion protein is found in approximately 12% of all AML patients. In this review, we detail the structural features, functions, and models used to study both RUNX1 and RUNX1-ETO in hematopoiesis over the past two decades.

PubMed Disclaimer

Figures

Figure 1
Figure 1
RUNX1 isoforms and genomic locus. (A) The RUNX1 genomic locus on chromosome 21 is shown with the location of the proximal and distal promotors and exons based on the National Center for Biotechnology Information Nucleotide database. (B) The three main transcriptional isoform of RUNX1 are shown. RUNX1a is consists of exons 4a through 8. RUNX1b consists of exons 4a through 10, but excludes exon 8. RUNX1c includes exons 1 through 3 and exons 4b through 10, but also excludes exon 8. (C) The three main RUNX1 isoforms are shown with the Runt homology domain shaded.
Figure 2
Figure 2
RUNX1 protein domains and interaction partners. The RUNX1b isoform is shown with the major domains listed above and interaction partners listed below.
Figure 3
Figure 3
RUNX1-ETO protein domains and interaction partners. The full length RUNX1-ETO is shown with the major domains listed above and interaction partners listed below.
See this image and copyright information in PMC

References

    1. van Wijnen AJ, Stein GS, Gergen JP, Groner Y, Hiebert SW, Ito Y, Liu P, Neil JC, Ohki M, Speck N. Nomenclature for Runt-related (RUNX) proteins. Oncogene. 2004;23:4209–4210. - PubMed
    1. Nussleinvolhard C, Wieschaus E. Mutations Affecting Segment Number and Polarity in Drosophila. Nature. 1980;287:795–801. - PubMed
    1. Kania MA, Bonner AS, Duffy JB, Gergen JP. The Drosophila Segmentation Gene Runt Encodes A Novel Nuclear Regulatory Protein That Is Also Expressed in the Developing Nervous-System. Genes & Development. 1990;4:1701–1713. - PubMed
    1. Kagoshima H, Shigesada K, Satake M, Ito Y, Miyoshi H, Ohki M, Pepling M, Gergen P. The Runt domain identifies a new family of heteromeric transcriptional regulators. Trends Genet. 1993;9:338–341. - PubMed
    1. Speck NA, Baltimore D. 6 Distinct Nuclear Factors Interact with the 75-Base-Pair Repeat of the Moloney Murine Leukemia-Virus Enhancer. Molecular and Cellular Biology. 1987;7:1101–1110. - PMC - PubMed

Publication types

MeSH terms

Substances