Role of sclerostin in mastocytosis bone disease

Abstract Mastocytosis is a heterogeneous group of disorders, characterized by accumulation of clonal mast cells which can infiltrate several organs, most often spine (70%). The pathogenesis of mastocytosis bone disease is poorly understood. The main aim of the study was to investigate whether neopla...

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Main Authors: Aneta Szudy-Szczyrek, Radosław Mlak, Dominika Pigoń-Zając, Witold Krupski, Marcin Mazurek, Aleksandra Tomczak, Karolina Chromik, Aleksandra Górska, Paweł Koźlik, Adrian Juda, Anna Kokoć, Maciej Dubaj, Tomasz Sacha, Marek Niedoszytko, Grzegorz Helbig, Michał Szczyrek, Justyna Szumiło, Teresa Małecka-Massalska, Marek Hus
Format: Article
Language:English
Published: Nature Portfolio 2025-01-01
Series:Scientific Reports
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Online Access:https://doi.org/10.1038/s41598-024-83851-0
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author Aneta Szudy-Szczyrek
Radosław Mlak
Dominika Pigoń-Zając
Witold Krupski
Marcin Mazurek
Aleksandra Tomczak
Karolina Chromik
Aleksandra Górska
Paweł Koźlik
Adrian Juda
Anna Kokoć
Maciej Dubaj
Tomasz Sacha
Marek Niedoszytko
Grzegorz Helbig
Michał Szczyrek
Justyna Szumiło
Teresa Małecka-Massalska
Marek Hus
author_facet Aneta Szudy-Szczyrek
Radosław Mlak
Dominika Pigoń-Zając
Witold Krupski
Marcin Mazurek
Aleksandra Tomczak
Karolina Chromik
Aleksandra Górska
Paweł Koźlik
Adrian Juda
Anna Kokoć
Maciej Dubaj
Tomasz Sacha
Marek Niedoszytko
Grzegorz Helbig
Michał Szczyrek
Justyna Szumiło
Teresa Małecka-Massalska
Marek Hus
author_sort Aneta Szudy-Szczyrek
collection DOAJ
description Abstract Mastocytosis is a heterogeneous group of disorders, characterized by accumulation of clonal mast cells which can infiltrate several organs, most often spine (70%). The pathogenesis of mastocytosis bone disease is poorly understood. The main aim of the study was to investigate whether neoplastic mast cells may be the source of sclerostin and whether there is an association between sclerostin and selected bone remodeling markers with mastocytosis related bone disease. We assessed sclerostin, bioactive sclerostin, and SOST gene expression in HMC-1.2 human mast cell culture supernatants and plasma of SM patients (n = 39). We showed that human mast cells can secrete sclerostin, and after their stimulation with IL-6, there is a significant increase in SOST gene expression. We observed significantly higher levels of sclerostin in patients diagnosed with more advanced disease. We observed a statistically significant correlation between concentations of sclerostin and its bioactive form and the concentration of alkaline phosphatase (ALP), and between sclerostin and interleukin-6 (IL-6). We observed that significantly higher sclerostin concentrations are present in patients with increased sclerosis of the spongy bone. Sclerostin may serve as a marker of more advanced disease and bone disease in mastocytosis. Further studies are justified to evaluate its role in mastocytosis.
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spelling doaj-art-440e0b52720940c1b345a50fca75327d2025-01-05T12:13:23ZengNature PortfolioScientific Reports2045-23222025-01-0115111110.1038/s41598-024-83851-0Role of sclerostin in mastocytosis bone diseaseAneta Szudy-Szczyrek0Radosław Mlak1Dominika Pigoń-Zając2Witold Krupski3Marcin Mazurek4Aleksandra Tomczak5Karolina Chromik6Aleksandra Górska7Paweł Koźlik8Adrian Juda9Anna Kokoć10Maciej Dubaj11Tomasz Sacha12Marek Niedoszytko13Grzegorz Helbig14Michał Szczyrek15Justyna Szumiło16Teresa Małecka-Massalska17Marek Hus18Department of Haematooncology and Bone Marrow Transplantation, Medical University of LublinDepartment of Laboratory Diagnostics, Medical University of LublinDepartment of Human Physiology, Medical University of LublinDepartment of Medical Radiology, Medical University of LublinDepartment of Human Physiology, Medical University of LublinDepartment of Haematooncology and Bone Marrow Transplantation, Medical University of LublinDepartment of Hematology and Bone Marrow Transplantation, Medical University of Silesia in KatowiceDepartment of Pulmonology and Allergology, Medical University of GdańskChair of Hematology, Jagiellonian UniversityDepartment of Haematooncology and Bone Marrow Transplantation, Medical University of LublinDepartment of Haematooncology and Bone Marrow Transplantation, Medical University of LublinDepartment of Haematooncology and Bone Marrow Transplantation, Medical University of LublinChair of Hematology, Jagiellonian UniversityDepartment of Pulmonology and Allergology, Medical University of GdańskDepartment of Hematology and Bone Marrow Transplantation, Medical University of Silesia in KatowiceDepartment of Pneumonology, Oncology and Allergology, Medical University of LublinDepartment of Clinical Pathomorphology, Medical University of LublinDepartment of Human Physiology, Medical University of LublinDepartment of Haematooncology and Bone Marrow Transplantation, Medical University of LublinAbstract Mastocytosis is a heterogeneous group of disorders, characterized by accumulation of clonal mast cells which can infiltrate several organs, most often spine (70%). The pathogenesis of mastocytosis bone disease is poorly understood. The main aim of the study was to investigate whether neoplastic mast cells may be the source of sclerostin and whether there is an association between sclerostin and selected bone remodeling markers with mastocytosis related bone disease. We assessed sclerostin, bioactive sclerostin, and SOST gene expression in HMC-1.2 human mast cell culture supernatants and plasma of SM patients (n = 39). We showed that human mast cells can secrete sclerostin, and after their stimulation with IL-6, there is a significant increase in SOST gene expression. We observed significantly higher levels of sclerostin in patients diagnosed with more advanced disease. We observed a statistically significant correlation between concentations of sclerostin and its bioactive form and the concentration of alkaline phosphatase (ALP), and between sclerostin and interleukin-6 (IL-6). We observed that significantly higher sclerostin concentrations are present in patients with increased sclerosis of the spongy bone. Sclerostin may serve as a marker of more advanced disease and bone disease in mastocytosis. Further studies are justified to evaluate its role in mastocytosis.https://doi.org/10.1038/s41598-024-83851-0Bone remodelingBonesMastocytosisOsteolysisOsteosclerosisSclerostin
spellingShingle Aneta Szudy-Szczyrek
Radosław Mlak
Dominika Pigoń-Zając
Witold Krupski
Marcin Mazurek
Aleksandra Tomczak
Karolina Chromik
Aleksandra Górska
Paweł Koźlik
Adrian Juda
Anna Kokoć
Maciej Dubaj
Tomasz Sacha
Marek Niedoszytko
Grzegorz Helbig
Michał Szczyrek
Justyna Szumiło
Teresa Małecka-Massalska
Marek Hus
Role of sclerostin in mastocytosis bone disease
Scientific Reports
Bone remodeling
Bones
Mastocytosis
Osteolysis
Osteosclerosis
Sclerostin
title Role of sclerostin in mastocytosis bone disease
title_full Role of sclerostin in mastocytosis bone disease
title_fullStr Role of sclerostin in mastocytosis bone disease
title_full_unstemmed Role of sclerostin in mastocytosis bone disease
title_short Role of sclerostin in mastocytosis bone disease
title_sort role of sclerostin in mastocytosis bone disease
topic Bone remodeling
Bones
Mastocytosis
Osteolysis
Osteosclerosis
Sclerostin
url https://doi.org/10.1038/s41598-024-83851-0
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