Targeting binding partners of the CBFβ-SMMHC fusion protein for the treatment of inversion 16 acute myeloid leukemia

Lisa Richter, Yiqian Wang and R. Katherine Hyde _

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Oncotarget. 2016; 7:66255-66266. https://doi.org/10.18632/oncotarget.11357

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Lisa Richter1,*, Yiqian Wang1,* and R. Katherine Hyde1

1 Department of Biochemistry and Molecular Biology and the Fred and Pamela Buffett Cancer Center, University of Nebraska Medical Center, Omaha, NE, USA

* These authors contributed equally to this work

Correspondence to:

R. Katherine Hyde, email:

Keywords: inv(16), CBFβ-SMMHC, CBFβ, RUNX1, AML

Received: March 14, 2016 Accepted: August 09, 2016 Published: August 17, 2016


Inversion of chromosome 16 (inv(16)) generates the CBFβ-SMMHC fusion protein and is found in nearly all patients with acute myeloid leukemia subtype M4 with Eosinophilia (M4Eo). Expression of CBFβ-SMMHC is causative for leukemia development, but the molecular mechanisms underlying its activity are unclear. Recently, there have been important advances in defining the role of CBFβ-SMMHC and its binding partners, the transcription factor RUNX1 and the histone deacetylase HDAC8. Importantly, initial trials demonstrate that small molecules targeting these binding partners are effective against CBFβ-SMMHC induced leukemia. This review will discuss recent advances in defining the mechanism of CBFβ-SMMHC activity, as well as efforts to develop new therapies for inv(16) AML.

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