The synergy of TPL and selinexor in MLL-R acute myeloid leukemia via Rap1/Raf/MEK pathway-mediated MYC downregulation

MLL gene rearrangement recurrently occurs in acute myeloid leukemia (MLL-r AML), which is closely associated with chemotherapy insensitivity and unfavorable clinical outcomes. More importantly, there are limited therapeutic options for the management of patients with MLL-r AML, thus necessitating no...

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Main Authors: Manman Deng, Peicui Huang, Lijuan Wang, Yuelong Jiang, Zhenling Guo, Hongpeng Duan, Jie Zha, Haijun Zhao, Guowei Li, Bing Xu
Format: Article
Language:English
Published: Elsevier 2025-07-01
Series:Translational Oncology
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Online Access:http://www.sciencedirect.com/science/article/pii/S1936523325001305
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author Manman Deng
Peicui Huang
Lijuan Wang
Yuelong Jiang
Zhenling Guo
Hongpeng Duan
Jie Zha
Haijun Zhao
Guowei Li
Bing Xu
author_facet Manman Deng
Peicui Huang
Lijuan Wang
Yuelong Jiang
Zhenling Guo
Hongpeng Duan
Jie Zha
Haijun Zhao
Guowei Li
Bing Xu
author_sort Manman Deng
collection DOAJ
description MLL gene rearrangement recurrently occurs in acute myeloid leukemia (MLL-r AML), which is closely associated with chemotherapy insensitivity and unfavorable clinical outcomes. More importantly, there are limited therapeutic options for the management of patients with MLL-r AML, thus necessitating novel effective treatment strategies. In this study, we demonstrated that low doses of triptolide (LD TPL) and the XPO1 inhibitor selinexor exerted synergistic therapeutic effects on poor-outcome MLL-r AML in vitro, ex vivo and in vivo. Induction of mitochondrial outer membrane permeabilization (MOMP) and initiation of the mitochondrial apoptotic pathway were closely involved in the therapeutic synergy of LD TPL in combination with selinexor against MLL-r AML. Mechanistically, MYC downregulation mediated by the Rap1/Raf/MEK/ERK pathway rather than by PI3K/AKT signaling was implicated in the synergistic activity of the combined regimen. In addition, the induction of DNA damage also contributed to the synergistic effects of the combined regimen on MLL-r AML. In summary, our findings suggest that LD TPL in combination with selinexor might represent a promising therapeutic approach for the treatment of MLL-r AML. However, future clinical trials are mandatory to draw a decisive conclusion.
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spelling doaj-art-6b2106fba2af4aebb3ac3edb7cddcaae2025-08-20T03:08:55ZengElsevierTranslational Oncology1936-52332025-07-015710239910.1016/j.tranon.2025.102399The synergy of TPL and selinexor in MLL-R acute myeloid leukemia via Rap1/Raf/MEK pathway-mediated MYC downregulationManman Deng0Peicui Huang1Lijuan Wang2Yuelong Jiang3Zhenling Guo4Hongpeng Duan5Jie Zha6Haijun Zhao7Guowei Li8Bing Xu9Department of Hematology, The First Affiliated Hospital of Xiamen University and Institute of Hematology, School of Medicine, Xiamen University, Xiamen, 361003, PR China; Key Laboratory of Xiamen for Diagnosis and Treatment of Hematological Malignancy, Xiamen, 361102, ChinaDepartment of Hematology, Huizhou Municipal Central Hospital, Huizhou, 516001, PR ChinaDepartment of Emergency, Women and Children’s Hospital, School of Medicine, Xiamen University, Xiamen, ChinaDepartment of Hematology, The First Affiliated Hospital of Xiamen University and Institute of Hematology, School of Medicine, Xiamen University, Xiamen, 361003, PR China; Key Laboratory of Xiamen for Diagnosis and Treatment of Hematological Malignancy, Xiamen, 361102, ChinaDepartment of Hematology, Huizhou Municipal Central Hospital, Huizhou, 516001, PR ChinaDepartment of Hematology, The First Affiliated Hospital of Xiamen University and Institute of Hematology, School of Medicine, Xiamen University, Xiamen, 361003, PR China; Key Laboratory of Xiamen for Diagnosis and Treatment of Hematological Malignancy, Xiamen, 361102, ChinaDepartment of Hematology, The First Affiliated Hospital of Xiamen University and Institute of Hematology, School of Medicine, Xiamen University, Xiamen, 361003, PR China; Key Laboratory of Xiamen for Diagnosis and Treatment of Hematological Malignancy, Xiamen, 361102, ChinaDepartment of Hematology, The First Affiliated Hospital of Xiamen University and Institute of Hematology, School of Medicine, Xiamen University, Xiamen, 361003, PR China; Key Laboratory of Xiamen for Diagnosis and Treatment of Hematological Malignancy, Xiamen, 361102, China; Corresponding authors at: Department of Hematology, the First Affiliated Hospital of Xiamen University and Institute of Hematology, Medical College of Xiamen University, No.55, Zhenhai Road, Siming District, Xiamen, Fujian 361003, China.Department of Hematology, Huizhou Municipal Central Hospital, Huizhou, 516001, PR China; Corresponding author at: Department of Hematology, Huizhou Municipal Central Hospital, Huizhou, 516001, PR China.Department of Hematology, The First Affiliated Hospital of Xiamen University and Institute of Hematology, School of Medicine, Xiamen University, Xiamen, 361003, PR China; Key Laboratory of Xiamen for Diagnosis and Treatment of Hematological Malignancy, Xiamen, 361102, China; Corresponding authors at: Department of Hematology, the First Affiliated Hospital of Xiamen University and Institute of Hematology, Medical College of Xiamen University, No.55, Zhenhai Road, Siming District, Xiamen, Fujian 361003, China.MLL gene rearrangement recurrently occurs in acute myeloid leukemia (MLL-r AML), which is closely associated with chemotherapy insensitivity and unfavorable clinical outcomes. More importantly, there are limited therapeutic options for the management of patients with MLL-r AML, thus necessitating novel effective treatment strategies. In this study, we demonstrated that low doses of triptolide (LD TPL) and the XPO1 inhibitor selinexor exerted synergistic therapeutic effects on poor-outcome MLL-r AML in vitro, ex vivo and in vivo. Induction of mitochondrial outer membrane permeabilization (MOMP) and initiation of the mitochondrial apoptotic pathway were closely involved in the therapeutic synergy of LD TPL in combination with selinexor against MLL-r AML. Mechanistically, MYC downregulation mediated by the Rap1/Raf/MEK/ERK pathway rather than by PI3K/AKT signaling was implicated in the synergistic activity of the combined regimen. In addition, the induction of DNA damage also contributed to the synergistic effects of the combined regimen on MLL-r AML. In summary, our findings suggest that LD TPL in combination with selinexor might represent a promising therapeutic approach for the treatment of MLL-r AML. However, future clinical trials are mandatory to draw a decisive conclusion.http://www.sciencedirect.com/science/article/pii/S1936523325001305MLL rearrangement (MLL-r)Acute myeloid leukemia (AML)TriptolideXPO1 inhibitor selinexorSynergyMYC
spellingShingle Manman Deng
Peicui Huang
Lijuan Wang
Yuelong Jiang
Zhenling Guo
Hongpeng Duan
Jie Zha
Haijun Zhao
Guowei Li
Bing Xu
The synergy of TPL and selinexor in MLL-R acute myeloid leukemia via Rap1/Raf/MEK pathway-mediated MYC downregulation
Translational Oncology
MLL rearrangement (MLL-r)
Acute myeloid leukemia (AML)
Triptolide
XPO1 inhibitor selinexor
Synergy
MYC
title The synergy of TPL and selinexor in MLL-R acute myeloid leukemia via Rap1/Raf/MEK pathway-mediated MYC downregulation
title_full The synergy of TPL and selinexor in MLL-R acute myeloid leukemia via Rap1/Raf/MEK pathway-mediated MYC downregulation
title_fullStr The synergy of TPL and selinexor in MLL-R acute myeloid leukemia via Rap1/Raf/MEK pathway-mediated MYC downregulation
title_full_unstemmed The synergy of TPL and selinexor in MLL-R acute myeloid leukemia via Rap1/Raf/MEK pathway-mediated MYC downregulation
title_short The synergy of TPL and selinexor in MLL-R acute myeloid leukemia via Rap1/Raf/MEK pathway-mediated MYC downregulation
title_sort synergy of tpl and selinexor in mll r acute myeloid leukemia via rap1 raf mek pathway mediated myc downregulation
topic MLL rearrangement (MLL-r)
Acute myeloid leukemia (AML)
Triptolide
XPO1 inhibitor selinexor
Synergy
MYC
url http://www.sciencedirect.com/science/article/pii/S1936523325001305
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