Bleximenib, the novel menin-KMT2A inhibitor JNJ-75276617, impairs long-term proliferation and immune evasion in acute myeloid leukemia

Acute myeloid leukemia (AML) remains challenging to treat, which in part relates to genetic heterogeneity of the disease, to the protective tumor microenvironment driving resistance to therapy, and also to immune evasion characteristics of leukemic cells. Targeting epigenetic programs in AML provid...

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Main Authors: Shanna M. Hogeling, Duy Minh Lê, Nikita La Rose, Min Chul Kwon, Albertus T.J. Wierenga, Fiona A.J. van den Heuvel, Vincent van den Boom, Anna Kuchnio, Ulrike Philippar, Gerwin Huls, Jan Jacob Schuringa
Format: Article
Language:English
Published: Ferrata Storti Foundation 2024-12-01
Series:Haematologica
Online Access:https://haematologica.org/article/view/11876
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author Shanna M. Hogeling
Duy Minh Lê
Nikita La Rose
Min Chul Kwon
Albertus T.J. Wierenga
Fiona A.J. van den Heuvel
Vincent van den Boom
Anna Kuchnio
Ulrike Philippar
Gerwin Huls
Jan Jacob Schuringa
author_facet Shanna M. Hogeling
Duy Minh Lê
Nikita La Rose
Min Chul Kwon
Albertus T.J. Wierenga
Fiona A.J. van den Heuvel
Vincent van den Boom
Anna Kuchnio
Ulrike Philippar
Gerwin Huls
Jan Jacob Schuringa
author_sort Shanna M. Hogeling
collection DOAJ
description Acute myeloid leukemia (AML) remains challenging to treat, which in part relates to genetic heterogeneity of the disease, to the protective tumor microenvironment driving resistance to therapy, and also to immune evasion characteristics of leukemic cells. Targeting epigenetic programs in AML provides an attractive opportunity to impair long-term proliferation and induce differentiation. The novel inhibitor JNJ- 75276617 (bleximenib) targets the menin-KMT2A interaction and provides preclinical efficacy in AML (Kwon et al1). Here, we provide mechanistic insight in how JNJ- 75276617 impairs proliferation and drives differentiation of primary AML patient cells. A large-scale drug screen was set up in which genetic alterations and quantitative proteomics were compared with drug sensitivity in a preclinical setting, which revealed that granulocyte macrophage progenitor (GMP)-like AMLs display the greatest sensitivity. Furthermore, we identified that NPM1c/DNMT3Amut AMLs are sensitive, and some NPM1wt AML subtypes without KMT2A-MLLT3 rearrangements benefit from menin-KMT2A inhibition. Genome-wide ChIP-seq studies revealed patient-specific epigenetic alterations upon JNJ-75276617 treatment, uncovering a striking upregulation of MHC class I and class II expression as a consequence of epigenetic changes upon menin-KMT2A inhibition, independent of MEIS1 loss but involving CIITA activation. Functionally, this results in enhanced sensitivity of leukemic blasts to T cell-mediated cytotoxicity in allogeneic and autologous settings. Our data indicate that JNJ-75276617 provides a potential therapeutic approach whereby not only proliferation is impaired and differentiation is induced, but whereby therapeutic benefit might also be achieved by reactivating the antigen presentation machinery.
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spelling doaj-art-b12e34c2d21c432d86cb3d9b8a8be1382024-12-19T19:43:19ZengFerrata Storti FoundationHaematologica0390-60781592-87212024-12-01999110.3324/haematol.2024.285616Bleximenib, the novel menin-KMT2A inhibitor JNJ-75276617, impairs long-term proliferation and immune evasion in acute myeloid leukemiaShanna M. Hogeling0Duy Minh Lê1Nikita La Rose2Min Chul Kwon3Albertus T.J. Wierenga4Fiona A.J. van den Heuvel5Vincent van den Boom6Anna Kuchnio7Ulrike Philippar8Gerwin Huls9Jan Jacob Schuringa10Department of Hematology, University Medical Center Groningen, University of Groningen, GroningenDepartment of Hematology, University Medical Center Groningen, University of Groningen, GroningenDepartment of Hematology, University Medical Center Groningen, University of Groningen, GroningenDiscovery Oncology, Janssen RD, BeerseDepartment of Hematology, University Medical Center Groningen, University of Groningen, GroningenDepartment of Hematology, University Medical Center Groningen, University of Groningen, GroningenDepartment of Hematology, University Medical Center Groningen, University of Groningen, GroningenDiscovery Oncology, Janssen RD, BeerseDiscovery Oncology, Janssen RD, BeerseDepartment of Hematology, University Medical Center Groningen, University of Groningen, GroningenDepartment of Hematology, University Medical Center Groningen, University of Groningen, Groningen Acute myeloid leukemia (AML) remains challenging to treat, which in part relates to genetic heterogeneity of the disease, to the protective tumor microenvironment driving resistance to therapy, and also to immune evasion characteristics of leukemic cells. Targeting epigenetic programs in AML provides an attractive opportunity to impair long-term proliferation and induce differentiation. The novel inhibitor JNJ- 75276617 (bleximenib) targets the menin-KMT2A interaction and provides preclinical efficacy in AML (Kwon et al1). Here, we provide mechanistic insight in how JNJ- 75276617 impairs proliferation and drives differentiation of primary AML patient cells. A large-scale drug screen was set up in which genetic alterations and quantitative proteomics were compared with drug sensitivity in a preclinical setting, which revealed that granulocyte macrophage progenitor (GMP)-like AMLs display the greatest sensitivity. Furthermore, we identified that NPM1c/DNMT3Amut AMLs are sensitive, and some NPM1wt AML subtypes without KMT2A-MLLT3 rearrangements benefit from menin-KMT2A inhibition. Genome-wide ChIP-seq studies revealed patient-specific epigenetic alterations upon JNJ-75276617 treatment, uncovering a striking upregulation of MHC class I and class II expression as a consequence of epigenetic changes upon menin-KMT2A inhibition, independent of MEIS1 loss but involving CIITA activation. Functionally, this results in enhanced sensitivity of leukemic blasts to T cell-mediated cytotoxicity in allogeneic and autologous settings. Our data indicate that JNJ-75276617 provides a potential therapeutic approach whereby not only proliferation is impaired and differentiation is induced, but whereby therapeutic benefit might also be achieved by reactivating the antigen presentation machinery. https://haematologica.org/article/view/11876
spellingShingle Shanna M. Hogeling
Duy Minh Lê
Nikita La Rose
Min Chul Kwon
Albertus T.J. Wierenga
Fiona A.J. van den Heuvel
Vincent van den Boom
Anna Kuchnio
Ulrike Philippar
Gerwin Huls
Jan Jacob Schuringa
Bleximenib, the novel menin-KMT2A inhibitor JNJ-75276617, impairs long-term proliferation and immune evasion in acute myeloid leukemia
Haematologica
title Bleximenib, the novel menin-KMT2A inhibitor JNJ-75276617, impairs long-term proliferation and immune evasion in acute myeloid leukemia
title_full Bleximenib, the novel menin-KMT2A inhibitor JNJ-75276617, impairs long-term proliferation and immune evasion in acute myeloid leukemia
title_fullStr Bleximenib, the novel menin-KMT2A inhibitor JNJ-75276617, impairs long-term proliferation and immune evasion in acute myeloid leukemia
title_full_unstemmed Bleximenib, the novel menin-KMT2A inhibitor JNJ-75276617, impairs long-term proliferation and immune evasion in acute myeloid leukemia
title_short Bleximenib, the novel menin-KMT2A inhibitor JNJ-75276617, impairs long-term proliferation and immune evasion in acute myeloid leukemia
title_sort bleximenib the novel menin kmt2a inhibitor jnj 75276617 impairs long term proliferation and immune evasion in acute myeloid leukemia
url https://haematologica.org/article/view/11876
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