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Modelling acquired resistance to DOT1L inhibition exhibits the adaptive potential of KMT2A-rearranged acute lymphoblastic leukemia

Abstract:

In KMT2A-rearranged acute lymphoblastic leukemia (ALL), an aggressive malignancy, oncogenic KMT2A-fusion proteins inappropriately recruit DOT1L to promote leukemogenesis, highlighting DOT1L as an attractive therapeutic target. Unfortunately, treatment with the first-in-class DOT1L inhibitor pinometostat eventually leads to non-responsiveness. To understand this we established acquired pinometostat resistance in pediatric KMT2A::AFF1+ B-ALL cells. Interestingly, these cells became mostly independent of DOT1L-mediated H3K79 methylation, but still relied on the physical presence of DOT1L, HOXA9 and the KMT2A::AFF1 fusion. Moreover, these cells selectively lost the epigenetic regulation and expression of various KMT2A-fusion target genes such as PROM1/CD133, while other KMT2A::AFF1 target genes, including HOXA9 and CDK6 remained unaffected. Concomitantly, these pinometostat-resistant cells showed upregulation of several myeloid-associated genes, including CD33 and LILRB4/CD85k. Taken together, this model comprehensively shows the adaptive potential of KMT2A-rearranged ALL cells upon losing dependency on one of its main oncogenic properties.

Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1186/s40164-023-00445-8

Authors


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Role:
Author
ORCID:
0000-0001-9610-6763


Publisher:
BioMed Central
Journal:
Experimental Hematology and Oncology More from this journal
Volume:
12
Issue:
1
Article number:
81
Publication date:
2023-09-22
Acceptance date:
2023-09-11
DOI:
EISSN:
2162-3619
Pmid:
37740239


Language:
English
Keywords:
Pubs id:
1537307
Local pid:
pubs:1537307
Deposit date:
2024-01-06

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