The Sec61 translocon is a therapeutic vulnerability in multiple myeloma

Abstract Multiple myeloma (MM) is an incurable malignancy characterized by the uncontrolled expansion of plasma cells in the bone marrow. While proteasome inhibitors like bortezomib efficiently halt MM progression, drug resistance inevitably develop, and novel therapeutic approaches are needed. Here...

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Main Authors: Antoine Domenger, Caroline Choisy, Ludivine Baron, Véronique Mayau, Emeline Perthame, Ludovic Deriano, Bertrand Arnulf, Jean‐Christophe Bories, Gilles Dadaglio, Caroline Demangel
Format: Article
Language:English
Published: Springer Nature 2022-01-01
Series:EMBO Molecular Medicine
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Online Access:https://doi.org/10.15252/emmm.202114740
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author Antoine Domenger
Caroline Choisy
Ludivine Baron
Véronique Mayau
Emeline Perthame
Ludovic Deriano
Bertrand Arnulf
Jean‐Christophe Bories
Gilles Dadaglio
Caroline Demangel
author_facet Antoine Domenger
Caroline Choisy
Ludivine Baron
Véronique Mayau
Emeline Perthame
Ludovic Deriano
Bertrand Arnulf
Jean‐Christophe Bories
Gilles Dadaglio
Caroline Demangel
author_sort Antoine Domenger
collection DOAJ
description Abstract Multiple myeloma (MM) is an incurable malignancy characterized by the uncontrolled expansion of plasma cells in the bone marrow. While proteasome inhibitors like bortezomib efficiently halt MM progression, drug resistance inevitably develop, and novel therapeutic approaches are needed. Here, we used a recently discovered Sec61 inhibitor, mycolactone, to assess the interest of disrupting MM proteostasis via protein translocation blockade. In human MM cell lines, mycolactone caused rapid defects in secretion of immunoglobulins and expression of pro‐survival interleukin (IL)‐6 receptor and CD40, whose activation stimulates IL‐6 production. Mycolactone also triggered pro‐apoptotic endoplasmic reticulum stress responses synergizing with bortezomib for induction of MM cell death and overriding acquired resistance to the proteasome inhibitor. Notably, the mycolactone–bortezomib combination rapidly killed patient‐derived MM cells ex vivo, but not normal mononuclear cells. In immunodeficient mice engrafted with MM cells, it demonstrated superior therapeutic efficacy over single drug treatments, without inducing toxic side effects. Collectively, these findings establish Sec61 blockers as novel anti‐MM agents and reveal the interest of targeting both the translocon and the proteasome in proteostasis‐addicted tumors.
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spelling doaj-art-22da9be175514a33a0ab22c7d255aaf82025-08-20T04:03:07ZengSpringer NatureEMBO Molecular Medicine1757-46761757-46842022-01-0114311710.15252/emmm.202114740The Sec61 translocon is a therapeutic vulnerability in multiple myelomaAntoine Domenger0Caroline Choisy1Ludivine Baron2Véronique Mayau3Emeline Perthame4Ludovic Deriano5Bertrand Arnulf6Jean‐Christophe Bories7Gilles Dadaglio8Caroline Demangel9Unité d’Immunobiologie de l’Infection, Institut Pasteur, INSERM U1224, Université de ParisINSERM U976, Institut de Recherche Saint Louis, Université de ParisUnité d’Immunobiologie de l’Infection, Institut Pasteur, INSERM U1224, Université de ParisUnité d’Immunobiologie de l’Infection, Institut Pasteur, INSERM U1224, Université de ParisBioinformatics and Biostatistics Hub, Institut Pasteur, Université de ParisUnité d’Intégrité du Génome, Immunité et Cancer, Equipe Labellisée Ligue Contre Le Cancer, Institut Pasteur, INSERM U1223, Université de ParisINSERM U976, Institut de Recherche Saint Louis, Université de ParisINSERM U976, Institut de Recherche Saint Louis, Université de ParisUnité d’Immunobiologie de l’Infection, Institut Pasteur, INSERM U1224, Université de ParisUnité d’Immunobiologie de l’Infection, Institut Pasteur, INSERM U1224, Université de ParisAbstract Multiple myeloma (MM) is an incurable malignancy characterized by the uncontrolled expansion of plasma cells in the bone marrow. While proteasome inhibitors like bortezomib efficiently halt MM progression, drug resistance inevitably develop, and novel therapeutic approaches are needed. Here, we used a recently discovered Sec61 inhibitor, mycolactone, to assess the interest of disrupting MM proteostasis via protein translocation blockade. In human MM cell lines, mycolactone caused rapid defects in secretion of immunoglobulins and expression of pro‐survival interleukin (IL)‐6 receptor and CD40, whose activation stimulates IL‐6 production. Mycolactone also triggered pro‐apoptotic endoplasmic reticulum stress responses synergizing with bortezomib for induction of MM cell death and overriding acquired resistance to the proteasome inhibitor. Notably, the mycolactone–bortezomib combination rapidly killed patient‐derived MM cells ex vivo, but not normal mononuclear cells. In immunodeficient mice engrafted with MM cells, it demonstrated superior therapeutic efficacy over single drug treatments, without inducing toxic side effects. Collectively, these findings establish Sec61 blockers as novel anti‐MM agents and reveal the interest of targeting both the translocon and the proteasome in proteostasis‐addicted tumors.https://doi.org/10.15252/emmm.202114740multiple myelomaproteostatic stressSec61 translocon
spellingShingle Antoine Domenger
Caroline Choisy
Ludivine Baron
Véronique Mayau
Emeline Perthame
Ludovic Deriano
Bertrand Arnulf
Jean‐Christophe Bories
Gilles Dadaglio
Caroline Demangel
The Sec61 translocon is a therapeutic vulnerability in multiple myeloma
EMBO Molecular Medicine
multiple myeloma
proteostatic stress
Sec61 translocon
title The Sec61 translocon is a therapeutic vulnerability in multiple myeloma
title_full The Sec61 translocon is a therapeutic vulnerability in multiple myeloma
title_fullStr The Sec61 translocon is a therapeutic vulnerability in multiple myeloma
title_full_unstemmed The Sec61 translocon is a therapeutic vulnerability in multiple myeloma
title_short The Sec61 translocon is a therapeutic vulnerability in multiple myeloma
title_sort sec61 translocon is a therapeutic vulnerability in multiple myeloma
topic multiple myeloma
proteostatic stress
Sec61 translocon
url https://doi.org/10.15252/emmm.202114740
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