Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Runx1

  • Amy C. Moore
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_5131-2

Synonyms

Definition

The RUNX1/CBFβ heterodimeric transcription factor regulates the expression of genes required for hematopoiesis and is one of the most frequent targets of chromosomal translocations associated with human leukemia.

Characteristics

Expression and Regulation

RUNX1 was originally isolated from acute myeloid leukemia patient samples containing the t(8;21) chromosomal translocation. In addition to RUNX1, mammals also express RUNX2 and RUNX3. All three RUNX proteins share homology with the Drosophila Runt protein and contain the 128 amino acid Runt homology domain (RHD), which mediates both DNA binding and interaction with the core binding factor-β (CBFβ) protein. Through this interaction, CBFβ stabilizes RUNX-DNA binding and protects the RUNX subunit from degradation via ubiquitination.

RUNX1maps to chromosome 21, spans 260 kb, and contains 11 exons, with...

Keywords

Acute Myeloid Leukemia Chromosomal Translocation Transcriptional Corepressor Smooth Muscle Myosin Heavy Chain RUNX1 Activity 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Engel M, Hiebert SW (2010) Pro-leukemic RUNX1 and CBFβ mutations in the pathogenesis of acute leukemia. Cancer Treat Res 145:127-147Google Scholar
  2. Kurokawa M, Hirai H (2003) Role of AML1/Runx1 in the pathogenesis of hematological malignancies. Cancer Sci 94:841–846CrossRefPubMedGoogle Scholar
  3. Levanon D, Groner Y (2004) Structure and regulated expression of mammalian RUNX genes. Oncogene 23:4211–4219CrossRefPubMedGoogle Scholar
  4. Miyoshi H, Shimizu K, Kozu T et al (1991) t(8;21) breakpoints on chromosome 21 in acute myeloid leukemia are clustered within a limited region of a single gene, AML1. Proc Natl Acad Sci U S A 88:10431–10434CrossRefPubMedPubMedCentralGoogle Scholar
  5. Okuda T, van Deursen J, Hiebert SW et al (1996) AML1, the target of multiple chromosomal translocations in human leukemia, is essential for normal fetal liver hematopoiesis. Cell 84:321–330CrossRefPubMedGoogle Scholar

See Also

  1. (2012) AML-1/ETO/CBFβ/TEL in Chromosomal Translocations. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 157. doi: 10.1007/978-3-642-16483-5_232Google Scholar
  2. (2012) C/EBPα. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 720. doi: 10.1007/978-3-642-16483-5_980Google Scholar
  3. (2012) HDACs. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 1635. doi: 10.1007/978-3-642-16483-5_2592Google Scholar
  4. (2012) Hematopoiesis. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 1644. doi: 10.1007/978-3-642-16483-5_2616Google Scholar
  5. (2012) Leukemia. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 2005. doi: 10.1007/978-3-642-16483-5_3322Google Scholar
  6. (2012) Self-Renewal. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 3359. doi: 10.1007/978-3-642-16483-5_5226Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Georgia Cancer CoalitionAtlantaUSA