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Research Papers:

S55746 is a novel orally active BCL-2 selective and potent inhibitor that impairs hematological tumor growth

Patrick Casara, James Davidson, Audrey Claperon, Gaëtane Le Toumelin-Braizat, Meike Vogler, Alain Bruno, Maïa Chanrion, Gaëlle Lysiak-Auvity, Thierry Le Diguarher, Jérôme-Benoît Starck, Ijen Chen, Neil Whitehead, Christopher Graham, Natalia Matassova, Pawel Dokurno, Christopher Pedder, Youzhen Wang, Shumei Qiu, Anne-Marie Girard, Emilie Schneider, Fabienne Gravé, Aurélie Studeny, Ghislaine Guasconi, Francesca Rocchetti, Sophie Maïga, Jean-Michel Henlin, Frédéric Colland, Laurence Kraus-Berthier, Steven Le Gouill, Martin J.S. Dyer, Roderick Hubbard, Mike Wood, Martine Amiot, Gerald M. Cohen, John A. Hickman, Erick Morris, James Murray and Olivier Geneste _

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Oncotarget. 2018; 9:20075-20088. https://doi.org/10.18632/oncotarget.24744

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Abstract

Patrick Casara1,*, James Davidson2,*, Audrey Claperon3,*, Gaëtane Le Toumelin-Braizat3, Meike Vogler4, Alain Bruno5, Maïa Chanrion3, Gaëlle Lysiak-Auvity3, Thierry Le Diguarher1, Jérôme-Benoît Starck1, Ijen Chen2, Neil Whitehead2, Christopher Graham2, Natalia Matassova2, Pawel Dokurno2, Christopher Pedder2, Youzhen Wang6, Shumei Qiu6, Anne-Marie Girard3, Emilie Schneider3, Fabienne Gravé3, Aurélie Studeny3, Ghislaine Guasconi3, Francesca Rocchetti3, Sophie Maïga7, Jean-Michel Henlin1, Frédéric Colland3, Laurence Kraus-Berthier5, Steven Le Gouill7, Martin J.S. Dyer8, Roderick Hubbard2, Mike Wood2, Martine Amiot7, Gerald M Cohen9, John A. Hickman3, Erick Morris6, James Murray2 and Olivier Geneste3

1Institut de Recherches Servier Discovery Chemistry Unit, Croissy Sur Seine, France

2Vernalis (R&D) Ltd., Cambridge, UK

3Institut de Recherches Servier Oncology R&D Unit, Croissy Sur Seine, France

4Institute for Experimental Cancer Research in Pediatrics, Goethe-University Frankfurt, Frankfurt, Germany

5Institut de Recherches Internationales Servier, Oncology R&D Unit, Suresnes, France

6Novartis Institute of Biomedical Research, Oncology Drug Discovery, Cambridge, MA, USA

7CRCINA, INSERM, CNRS, Université de Nantes, CHU de Nantes, Nantes, France

8Ernest and Helen Scott Haematological Research Institute, University of Leicester, Leicester, UK

9Institute of Translational Medicine, University of Liverpool, Liverpool, UK

*These authors contributed equally to this work

Correspondence to:

Olivier Geneste, email: [email protected]

Keywords: BCL-2; inhibitor; BH3-mimetics; apoptosis; hematological malignancies

Received: September 12, 2017     Accepted: February 26, 2018     Published: April 13, 2018

ABSTRACT

Escape from apoptosis is one of the major hallmarks of cancer cells. The B-cell Lymphoma 2 (BCL-2) gene family encodes pro-apoptotic and anti-apoptotic proteins that are key regulators of the apoptotic process. Overexpression of the pro-survival member BCL-2 is a well-established mechanism contributing to oncogenesis and chemoresistance in several cancers, including lymphoma and leukemia. Thus, BCL-2 has become an attractive target for therapeutic strategy in cancer, as demonstrated by the recent approval of ABT-199 (Venclexta™) in relapsed or refractory Chronic Lymphocytic Leukemia with 17p deletion. Here, we describe a novel orally bioavailable BCL-2 selective and potent inhibitor called S55746 (also known as BCL201). S55746 occupies the hydrophobic groove of BCL-2. Its selectivity profile demonstrates no significant binding to MCL-1, BFL-1 (BCL2A1/A1) and poor affinity for BCL-XL. Accordingly, S55746 has no cytotoxic activity on BCL-XL-dependent cells, such as platelets. In a panel of hematological cell lines, S55746 induces hallmarks of apoptosis including externalization of phosphatidylserine, caspase-3 activation and PARP cleavage. Ex vivo, S55746 induces apoptosis in the low nanomolar range in primary Chronic Lymphocytic Leukemia and Mantle Cell Lymphoma patient samples. Finally, S55746 administered by oral route daily in mice demonstrated robust anti-tumor efficacy in two hematological xenograft models with no weight lost and no change in behavior. Taken together, these data demonstrate that S55746 is a novel, well-tolerated BH3-mimetic targeting selectively and potently the BCL-2 protein.


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