Reactome | CBFB binds RUNX1

CBFB binds RUNX1

Stable Identifier

R-HSA-8865320

Type

Reaction [binding]

Species

Homo sapiens

Compartment

nucleoplasm

ReviewStatus

5/5

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The heterodimerization domain of CBFB binds to the Runt domain of RUNX1 (AML1) to form a RUNX1:CBFB heterodimer (Warren et al. 2000, Lukasik et al. 2002). Formation of the RUNX1:CBFB heterodimer was first demonstrated in Drosophila (Ogawa et al. 1993). While RUNX1 is the DNA binding subunit, the presence of CBFB is necessary for the transcriptional activity of the RUNX1:CBFB complex, based on knockout mouse studies (Wang et al. 1996).
The RUNX1:CBFB transcription complex is essential for hematopoiesis (Warren et al. 2000).

Both CBFB and RUNX1 are subject to frequent mutations in leukemia (Ustun and Marcucci 2015).

Literature References

PubMed ID	Title	Journal	Year
10856244	Structural basis for the heterodimeric interaction between the acute leukaemia-associated transcription factors AML1 and CBFbeta Warren, AJ, Bravo, J, Williams, RL, Rabbitts, TH	EMBO J.	2000
8386878	Molecular cloning and characterization of PEBP2 beta, the heterodimeric partner of a novel Drosophila runt-related DNA binding protein PEBP2 alpha Ogawa, E, Inuzuka, M, Maruyama, M, Satake, M, Naito-Fujimoto, M, Ito, Y, Shigesada, K	Virology	1993
12172539	Altered affinity of CBF beta-SMMHC for Runx1 explains its role in leukemogenesis Lukasik, SM, Zhang, L, Corpora, T, Tomanicek, S, Li, Y, Kundu, M, Hartman, K, Liu, PP, Laue, TM, Biltonen, RL, Speck, NA, Bushweller, JH	Nat. Struct. Biol.	2002