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2016 ; 7
(40
): 66255-66266
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Targeting binding partners of the CBF?-SMMHC fusion protein for the treatment of
inversion 16 acute myeloid leukemia
#MMPMID27542261
Richter L
; Wang Y
; Hyde RK
Oncotarget
2016[Oct]; 7
(40
): 66255-66266
PMID27542261
show ga
Inversion of chromosome 16 (inv(16)) generates the CBF?-SMMHC fusion protein and
is found in nearly all patients with acute myeloid leukemia subtype M4 with
Eosinophilia (M4Eo). Expression of CBF?-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 CBF?-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 CBF?-SMMHC induced
leukemia. This review will discuss recent advances in defining the mechanism of
CBF?-SMMHC activity, as well as efforts to develop new therapies for inv(16) AML.