The role of the extracellular matrix in primary myelofibrosis.
Primary myelofibrosis (PMF) is a myeloproliferative neoplasm that arises from clonal proliferation of hematopoietic stem cells and leads to progressive bone marrow (BM) fibrosis. While cellular mutations involved in the development of PMF have been heavily investigated, noteworthy is the important role the extracellular matrix (ECM) plays in the progression of BM fibrosis. This review surveys ECM proteins contributors of PMF, and highlights how better understanding of the control of the ECM within the BM niche may lead to combined therapeutic options in PMF.
Department of Medicine and Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston, MA, USA.
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Department of Medicine and Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston, MA, USA.
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Department of Medicine and Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston, MA, USA.
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Chitalia
Department of Molecular Medicine, University of Pavia, Pavia, Italy.|Laboratory of Biotechnology, IRCCS San Matteo Foundation, Pavia, Italy.
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Balduini
Department of Medicine and Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston, MA, USA.
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Matsuura
Department of Medicine and Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston, MA, USA.
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D005109:Extracellular Matrix / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D009154:Mutation; D055728:Primary Myelofibrosis / Q000235:genetics* / Q000473:pathology