The P2X7R-antagonist AFC-5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigm

BackgroundMultiple sclerosis (MS) is characterized by chronic inflammation driven by central nervous system (CNS)-resident immune cells such as microglia, especially during the progressive phase of the disease. The P2X7 receptor (P2X7R), a risk protein for MS, is ubiquitously expressed on immune cel...

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Main Authors: Robert Hoffrogge, Anna Karachunskaya, Neele Heitmann, Steffen Haupeltshofer, Xiomara Pedreiturria, Katharina Klöster, Verian Bader, Konstanze F. Winklhofer, Michael Hamacher, Bert Klebl, Ralf Gold, Klaus Dinkel, Ingo Kleiter, Simon Faissner
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Language:English
Published: Frontiers Media S.A. 2025-04-01
Series:Frontiers in Immunology
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Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2025.1554999/full
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author Robert Hoffrogge
Anna Karachunskaya
Neele Heitmann
Steffen Haupeltshofer
Xiomara Pedreiturria
Katharina Klöster
Verian Bader
Konstanze F. Winklhofer
Michael Hamacher
Bert Klebl
Ralf Gold
Klaus Dinkel
Ingo Kleiter
Ingo Kleiter
Simon Faissner
author_facet Robert Hoffrogge
Anna Karachunskaya
Neele Heitmann
Steffen Haupeltshofer
Xiomara Pedreiturria
Katharina Klöster
Verian Bader
Konstanze F. Winklhofer
Michael Hamacher
Bert Klebl
Ralf Gold
Klaus Dinkel
Ingo Kleiter
Ingo Kleiter
Simon Faissner
author_sort Robert Hoffrogge
collection DOAJ
description BackgroundMultiple sclerosis (MS) is characterized by chronic inflammation driven by central nervous system (CNS)-resident immune cells such as microglia, especially during the progressive phase of the disease. The P2X7 receptor (P2X7R), a risk protein for MS, is ubiquitously expressed on immune cells. AFC-5128, a CNS-penetrating small molecule inhibitor of P2X7R, is a promising agent for the treatment of autoimmune diseases such as MS.MethodsIn vitro, the effects on the calcium influx of primary murine microglia were assessed via Fluo-4 calcium imaging. In vivo, MOG35-55 immunized C57BL/6 mice were treated with AFC-5128, fingolimod (FTY) or vehicle in different treatment paradigms. The mice were scored daily. Microglial marker expression, immune cell phenotyping and serum cytokine analyses were performed via flow cytometry. Immune cell infiltration, demyelination and Iba1+/CD3+ cells were detected in spinal cord cross-sections. The effects of MOG35-55 T-cell restimulation were assessed in vitro.ResultsIn vitro, treatment of primary microglia with 10 µM AFC-5128 reduced the influx of calcium following ATP stimulation (p<0.0001). In vivo, treatment of mice with AFC-5128 led to a reduction in overall EAE scores in acute and chronic EAE, with the best effects using 200 mg/kg body weight AFC-5128 (p<0.0001). Peripheral immune cell subsets (B cells, T cells and macrophages) and serum cytokine levels of chronic EAE mice treated in a therapeutic paradigm were not affected. While the expression of homeostasis markers of microglia in AFC-5128-treated mice was not affected, there was a trend toward lower expression of phagocytosis-associated markers. Late therapeutic treatment with AFC-5128 had only mild effects on chronic EAE.ConclusionThe treatment of EAE mice with AFC-5128 improved acute and chronic EAE in different treatment paradigms, with positive effects on histological markers and slight modulation of microglial marker expression. Mechanistically, calcium influx of microglia was reduced following AFC-5128 treatment, which implies the ability of AFC-5128 to stabilize calcium homeostasis. Therefore, therapeutic inhibition of P2X7R via AFC-5128 has the potential for translation into a treatment of both relapsing and progressive forms of multiple sclerosis.
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spelling doaj-art-b3552ea34efe46129b6e78a0ba2fb8c12025-08-20T03:51:44ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-04-011610.3389/fimmu.2025.15549991554999The P2X7R-antagonist AFC-5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigmRobert Hoffrogge0Anna Karachunskaya1Neele Heitmann2Steffen Haupeltshofer3Xiomara Pedreiturria4Katharina Klöster5Verian Bader6Konstanze F. Winklhofer7Michael Hamacher8Bert Klebl9Ralf Gold10Klaus Dinkel11Ingo Kleiter12Ingo Kleiter13Simon Faissner14Department of Neurology, St. Josef-Hospital, Bochum, GermanyDepartment of Neurology, St. Josef-Hospital, Bochum, GermanyDepartment of Neurology, St. Josef-Hospital, Bochum, GermanyTranslational Neurology Group, Department of Clinical Science, Lund University, Lund, SwedenDepartment of Neurology, St. Josef-Hospital, Bochum, GermanyDepartment of Neurology, St. Josef-Hospital, Bochum, GermanyMolecular Cell Biology, Ruhr-University, Bochum, GermanyMolecular Cell Biology, Ruhr-University, Bochum, GermanyAffectis Pharmaceuticals AG, Dortmund, GermanyKHAN Technology Transfer Fund I GmbH & Co KG, Dortmund, GermanyDepartment of Neurology, St. Josef-Hospital, Bochum, GermanyLead Discovery Center GmbH, Dortmund, GermanyDepartment of Neurology, St. Josef-Hospital, Bochum, GermanyBehandlungszentrum Kempfenhausen für Multiple Sklerose Kranke gemeinnützige GmbH, Berg, GermanyDepartment of Neurology, St. Josef-Hospital, Bochum, GermanyBackgroundMultiple sclerosis (MS) is characterized by chronic inflammation driven by central nervous system (CNS)-resident immune cells such as microglia, especially during the progressive phase of the disease. The P2X7 receptor (P2X7R), a risk protein for MS, is ubiquitously expressed on immune cells. AFC-5128, a CNS-penetrating small molecule inhibitor of P2X7R, is a promising agent for the treatment of autoimmune diseases such as MS.MethodsIn vitro, the effects on the calcium influx of primary murine microglia were assessed via Fluo-4 calcium imaging. In vivo, MOG35-55 immunized C57BL/6 mice were treated with AFC-5128, fingolimod (FTY) or vehicle in different treatment paradigms. The mice were scored daily. Microglial marker expression, immune cell phenotyping and serum cytokine analyses were performed via flow cytometry. Immune cell infiltration, demyelination and Iba1+/CD3+ cells were detected in spinal cord cross-sections. The effects of MOG35-55 T-cell restimulation were assessed in vitro.ResultsIn vitro, treatment of primary microglia with 10 µM AFC-5128 reduced the influx of calcium following ATP stimulation (p<0.0001). In vivo, treatment of mice with AFC-5128 led to a reduction in overall EAE scores in acute and chronic EAE, with the best effects using 200 mg/kg body weight AFC-5128 (p<0.0001). Peripheral immune cell subsets (B cells, T cells and macrophages) and serum cytokine levels of chronic EAE mice treated in a therapeutic paradigm were not affected. While the expression of homeostasis markers of microglia in AFC-5128-treated mice was not affected, there was a trend toward lower expression of phagocytosis-associated markers. Late therapeutic treatment with AFC-5128 had only mild effects on chronic EAE.ConclusionThe treatment of EAE mice with AFC-5128 improved acute and chronic EAE in different treatment paradigms, with positive effects on histological markers and slight modulation of microglial marker expression. Mechanistically, calcium influx of microglia was reduced following AFC-5128 treatment, which implies the ability of AFC-5128 to stabilize calcium homeostasis. Therefore, therapeutic inhibition of P2X7R via AFC-5128 has the potential for translation into a treatment of both relapsing and progressive forms of multiple sclerosis.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1554999/fullP2X7Rprogressive multiple sclerosismicroglianeuroinflammationneuroprotection
spellingShingle Robert Hoffrogge
Anna Karachunskaya
Neele Heitmann
Steffen Haupeltshofer
Xiomara Pedreiturria
Katharina Klöster
Verian Bader
Konstanze F. Winklhofer
Michael Hamacher
Bert Klebl
Ralf Gold
Klaus Dinkel
Ingo Kleiter
Ingo Kleiter
Simon Faissner
The P2X7R-antagonist AFC-5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigm
Frontiers in Immunology
P2X7R
progressive multiple sclerosis
microglia
neuroinflammation
neuroprotection
title The P2X7R-antagonist AFC-5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigm
title_full The P2X7R-antagonist AFC-5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigm
title_fullStr The P2X7R-antagonist AFC-5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigm
title_full_unstemmed The P2X7R-antagonist AFC-5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigm
title_short The P2X7R-antagonist AFC-5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigm
title_sort p2x7r antagonist afc 5128 ameliorates chronic experimental autoimmune encephalomyelitis in a preventive and therapeutic paradigm
topic P2X7R
progressive multiple sclerosis
microglia
neuroinflammation
neuroprotection
url https://www.frontiersin.org/articles/10.3389/fimmu.2025.1554999/full
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