Efficacy of JAK1/2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signaling

Abstract Ruxolitinib is a potent JAK1/JAK2 inhibitor, approved for the treatment of primary myelofibrosis (PMF) patients based on the concept of inhibition of oncogenic signaling. However, the effect of ruxolitinib on JAK2-V617F allelic burden is modest, suggesting that inhibition of JAK2-V617F sign...

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Main Authors: Sivahari Prasad Gorantla, Michael Rassner, Kirstyn Anne Crossley, Tony Andreas Müller, Teresa Poggio, Shifa Khaja Saleem, Helen Kleinfelder, Sudheer Madan Mohan Gambheer, Cornelia Endres, Sabina Schaberg, Dominik Schmidt, Gerin Prince, Irene Gonzalez-Menendez, Detlef Bentrop, Rainer Trittler, Svetlana Rylova, Dietmar Pfeifer, Geoffroy Andrieux, Leticia Quintanilla-Martinez, Anna Lena Illert, Nikolas von Bubnoff, Robert Zeiser, Justus Duyster
Format: Article
Language:English
Published: Nature Portfolio 2025-05-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-025-60019-6
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author Sivahari Prasad Gorantla
Michael Rassner
Kirstyn Anne Crossley
Tony Andreas Müller
Teresa Poggio
Shifa Khaja Saleem
Helen Kleinfelder
Sudheer Madan Mohan Gambheer
Cornelia Endres
Sabina Schaberg
Dominik Schmidt
Gerin Prince
Irene Gonzalez-Menendez
Detlef Bentrop
Rainer Trittler
Svetlana Rylova
Dietmar Pfeifer
Geoffroy Andrieux
Leticia Quintanilla-Martinez
Anna Lena Illert
Nikolas von Bubnoff
Robert Zeiser
Justus Duyster
author_facet Sivahari Prasad Gorantla
Michael Rassner
Kirstyn Anne Crossley
Tony Andreas Müller
Teresa Poggio
Shifa Khaja Saleem
Helen Kleinfelder
Sudheer Madan Mohan Gambheer
Cornelia Endres
Sabina Schaberg
Dominik Schmidt
Gerin Prince
Irene Gonzalez-Menendez
Detlef Bentrop
Rainer Trittler
Svetlana Rylova
Dietmar Pfeifer
Geoffroy Andrieux
Leticia Quintanilla-Martinez
Anna Lena Illert
Nikolas von Bubnoff
Robert Zeiser
Justus Duyster
author_sort Sivahari Prasad Gorantla
collection DOAJ
description Abstract Ruxolitinib is a potent JAK1/JAK2 inhibitor, approved for the treatment of primary myelofibrosis (PMF) patients based on the concept of inhibition of oncogenic signaling. However, the effect of ruxolitinib on JAK2-V617F allelic burden is modest, suggesting that inhibition of JAK2-V617F signaling-driven clone expansion is not the main mechanism of action. We evaluate whether ruxolitinib mainly blocks the proliferation of the malignant clone or exerts its effects also by targeting non-malignant cells. Therefore, we develop two JAK2-V617F-driven myeloproliferative neoplasm (MPN) mouse models harboring ruxolitinib resistance mutations. Mice carrying ruxolitinib-resistant JAK2-V617F-driven MPN respond to ruxolitinib treatment similar to mice with ruxolitinib-sensitive JAK2-V617F MPN with respect to reduction of spleen size, leukocyte count and pro-inflammatory cytokines in the serum. Ruxolitinib reduces pro-inflammatory cytokines in both stromal cells and non-malignant hematopoietic cells. Using a rigorous ruxolitinib resistance mutation approach, we can prove that ruxolitinib acts independent of oncogenic JAK2-V617F signaling and reduces the main features of MPN disease such as spleen size and leukocyte counts. Our findings characterize the mechanism of action for ruxolitinib in MPN.
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spelling doaj-art-db668830e3404d6b98b3f64d80bb55c92025-08-20T03:48:18ZengNature PortfolioNature Communications2041-17232025-05-0116111610.1038/s41467-025-60019-6Efficacy of JAK1/2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signalingSivahari Prasad Gorantla0Michael Rassner1Kirstyn Anne Crossley2Tony Andreas Müller3Teresa Poggio4Shifa Khaja Saleem5Helen Kleinfelder6Sudheer Madan Mohan Gambheer7Cornelia Endres8Sabina Schaberg9Dominik Schmidt10Gerin Prince11Irene Gonzalez-Menendez12Detlef Bentrop13Rainer Trittler14Svetlana Rylova15Dietmar Pfeifer16Geoffroy Andrieux17Leticia Quintanilla-Martinez18Anna Lena Illert19Nikolas von Bubnoff20Robert Zeiser21Justus Duyster22Faculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgDepartment of Hematology and Oncology, University Medical Center Schleswig-Holstein, and University Cancer Center Schleswig-HolsteinDepartment of Pathology and Neuropathology, University Hospital Tübingen & Comprehensive Cancer Center TübingenInstitute of Physiology, Albert-Ludwigs-Universität FreiburgInstitute of Pharmaceutical Sciences, Albert-Ludwigs-Universität FreiburgFaculty of Medicine, Department of Nuclear Medicine, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgGerman Cancer Consortium (DKTK) and German Cancer Research Center (DKFZ)Department of Pathology and Neuropathology, University Hospital Tübingen & Comprehensive Cancer Center TübingenFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgDepartment of Hematology and Oncology, University Medical Center Schleswig-Holstein, and University Cancer Center Schleswig-HolsteinFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgFaculty of Medicine, Clinic for Internal Medicine I, Hematology, Oncology and Stem cell transplantation, University Medical Center FreiburgAbstract Ruxolitinib is a potent JAK1/JAK2 inhibitor, approved for the treatment of primary myelofibrosis (PMF) patients based on the concept of inhibition of oncogenic signaling. However, the effect of ruxolitinib on JAK2-V617F allelic burden is modest, suggesting that inhibition of JAK2-V617F signaling-driven clone expansion is not the main mechanism of action. We evaluate whether ruxolitinib mainly blocks the proliferation of the malignant clone or exerts its effects also by targeting non-malignant cells. Therefore, we develop two JAK2-V617F-driven myeloproliferative neoplasm (MPN) mouse models harboring ruxolitinib resistance mutations. Mice carrying ruxolitinib-resistant JAK2-V617F-driven MPN respond to ruxolitinib treatment similar to mice with ruxolitinib-sensitive JAK2-V617F MPN with respect to reduction of spleen size, leukocyte count and pro-inflammatory cytokines in the serum. Ruxolitinib reduces pro-inflammatory cytokines in both stromal cells and non-malignant hematopoietic cells. Using a rigorous ruxolitinib resistance mutation approach, we can prove that ruxolitinib acts independent of oncogenic JAK2-V617F signaling and reduces the main features of MPN disease such as spleen size and leukocyte counts. Our findings characterize the mechanism of action for ruxolitinib in MPN.https://doi.org/10.1038/s41467-025-60019-6
spellingShingle Sivahari Prasad Gorantla
Michael Rassner
Kirstyn Anne Crossley
Tony Andreas Müller
Teresa Poggio
Shifa Khaja Saleem
Helen Kleinfelder
Sudheer Madan Mohan Gambheer
Cornelia Endres
Sabina Schaberg
Dominik Schmidt
Gerin Prince
Irene Gonzalez-Menendez
Detlef Bentrop
Rainer Trittler
Svetlana Rylova
Dietmar Pfeifer
Geoffroy Andrieux
Leticia Quintanilla-Martinez
Anna Lena Illert
Nikolas von Bubnoff
Robert Zeiser
Justus Duyster
Efficacy of JAK1/2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signaling
Nature Communications
title Efficacy of JAK1/2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signaling
title_full Efficacy of JAK1/2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signaling
title_fullStr Efficacy of JAK1/2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signaling
title_full_unstemmed Efficacy of JAK1/2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signaling
title_short Efficacy of JAK1/2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signaling
title_sort efficacy of jak1 2 inhibition in murine myeloproliferative neoplasms is not mediated by targeting oncogenic signaling
url https://doi.org/10.1038/s41467-025-60019-6
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