Functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastoma

Abstract Hepatoblastoma is a rare pediatric liver malignancy usually treated with surgery and chemotherapy. To explore new treatment options for hepatoblastoma, drug screening was performed using six cell models established from aggressive hepatoblastoma tumors and healthy pediatric primary hepatocy...

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Main Authors: Ruth Nousiainen, Katja Eloranta, Jani Saarela, Antti Hassinen, Tamara J. Luck, Stefano Cairo, Emilie Indersie, Swapnil Potdar, Michaela J. Feodoroff, Jouko Lohi, Lassi Paavolainen, David B. Wilson, Vilja Pietiäinen, Markku Heikinheimo, Marjut Pihlajoki
Format: Article
Language:English
Published: Nature Portfolio 2025-04-01
Series:npj Precision Oncology
Online Access:https://doi.org/10.1038/s41698-025-00915-8
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author Ruth Nousiainen
Katja Eloranta
Jani Saarela
Antti Hassinen
Tamara J. Luck
Stefano Cairo
Emilie Indersie
Swapnil Potdar
Michaela J. Feodoroff
Jouko Lohi
Lassi Paavolainen
David B. Wilson
Vilja Pietiäinen
Markku Heikinheimo
Marjut Pihlajoki
author_facet Ruth Nousiainen
Katja Eloranta
Jani Saarela
Antti Hassinen
Tamara J. Luck
Stefano Cairo
Emilie Indersie
Swapnil Potdar
Michaela J. Feodoroff
Jouko Lohi
Lassi Paavolainen
David B. Wilson
Vilja Pietiäinen
Markku Heikinheimo
Marjut Pihlajoki
author_sort Ruth Nousiainen
collection DOAJ
description Abstract Hepatoblastoma is a rare pediatric liver malignancy usually treated with surgery and chemotherapy. To explore new treatment options for hepatoblastoma, drug screening was performed using six cell models established from aggressive hepatoblastoma tumors and healthy pediatric primary hepatocytes. Of the 527 screened compounds, 98 demonstrated cancer-selective activity in at least one hepatoblastoma model. The kinesin spindle protein (KSP) inhibitor filanesib was effective in all models and was further evaluated. Filanesib induced G2/M arrest and apoptosis in hepatoblastoma cells at concentrations tolerable to primary hepatocytes. Prominent nuclear fragmentation was observed in filanesib-treated hepatoblastoma cells. Genes participating in cell cycle regulation were noted to be differentially expressed after filanesib treatment. Filanesib reduced the rate of tumor growth in 4/5 hepatoblastoma mice models. One of these models showed complete growth arrest. Our results suggest that filanesib is a potential candidate for hepatoblastoma treatment and should be investigated in future clinical trials.
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series npj Precision Oncology
spelling doaj-art-5ac5578eeef4411f9cfd2f356a013c052025-08-20T02:19:58ZengNature Portfolionpj Precision Oncology2397-768X2025-04-019111810.1038/s41698-025-00915-8Functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastomaRuth Nousiainen0Katja Eloranta1Jani Saarela2Antti Hassinen3Tamara J. Luck4Stefano Cairo5Emilie Indersie6Swapnil Potdar7Michaela J. Feodoroff8Jouko Lohi9Lassi Paavolainen10David B. Wilson11Vilja Pietiäinen12Markku Heikinheimo13Marjut Pihlajoki14Pediatric Research Center, Children’s Hospital, University of Helsinki and Helsinki University HospitalPediatric Research Center, Children’s Hospital, University of Helsinki and Helsinki University HospitalInstitute for Molecular Medicine Finland (FIMM), Helsinki Institute of Life Science (HiLIFE), University of HelsinkiInstitute for Molecular Medicine Finland (FIMM), Helsinki Institute of Life Science (HiLIFE), University of HelsinkiInstitute for Molecular Medicine Finland (FIMM), Helsinki Institute of Life Science (HiLIFE), University of HelsinkiXenTechXenTechInstitute for Molecular Medicine Finland (FIMM), Helsinki Institute of Life Science (HiLIFE), University of HelsinkiInstitute for Molecular Medicine Finland (FIMM), Helsinki Institute of Life Science (HiLIFE), University of HelsinkiDepartment of Pathology, University of Helsinki and Helsinki University HospitalInstitute for Molecular Medicine Finland (FIMM), Helsinki Institute of Life Science (HiLIFE), University of HelsinkiDepartment of Developmental Biology, Washington University School of MedicineInstitute for Molecular Medicine Finland (FIMM), Helsinki Institute of Life Science (HiLIFE), University of HelsinkiPediatric Research Center, Children’s Hospital, University of Helsinki and Helsinki University HospitalPediatric Research Center, Children’s Hospital, University of Helsinki and Helsinki University HospitalAbstract Hepatoblastoma is a rare pediatric liver malignancy usually treated with surgery and chemotherapy. To explore new treatment options for hepatoblastoma, drug screening was performed using six cell models established from aggressive hepatoblastoma tumors and healthy pediatric primary hepatocytes. Of the 527 screened compounds, 98 demonstrated cancer-selective activity in at least one hepatoblastoma model. The kinesin spindle protein (KSP) inhibitor filanesib was effective in all models and was further evaluated. Filanesib induced G2/M arrest and apoptosis in hepatoblastoma cells at concentrations tolerable to primary hepatocytes. Prominent nuclear fragmentation was observed in filanesib-treated hepatoblastoma cells. Genes participating in cell cycle regulation were noted to be differentially expressed after filanesib treatment. Filanesib reduced the rate of tumor growth in 4/5 hepatoblastoma mice models. One of these models showed complete growth arrest. Our results suggest that filanesib is a potential candidate for hepatoblastoma treatment and should be investigated in future clinical trials.https://doi.org/10.1038/s41698-025-00915-8
spellingShingle Ruth Nousiainen
Katja Eloranta
Jani Saarela
Antti Hassinen
Tamara J. Luck
Stefano Cairo
Emilie Indersie
Swapnil Potdar
Michaela J. Feodoroff
Jouko Lohi
Lassi Paavolainen
David B. Wilson
Vilja Pietiäinen
Markku Heikinheimo
Marjut Pihlajoki
Functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastoma
npj Precision Oncology
title Functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastoma
title_full Functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastoma
title_fullStr Functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastoma
title_full_unstemmed Functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastoma
title_short Functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastoma
title_sort functional screening identifies kinesin spindle protein inhibitor filanesib as a potential treatment option for hepatoblastoma
url https://doi.org/10.1038/s41698-025-00915-8
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