细胞外基质在原发性骨髓纤维化中的作用。
The role of the extracellular matrix in primary myelofibrosis.
作者信息
Leiva O, Ng S K, Chitalia S, Balduini A, Matsuura S, Ravid K
机构信息
Department of Medicine and Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston, MA, USA.
Department of Molecular Medicine, University of Pavia, Pavia, Italy.
出版信息
Blood Cancer J. 2017 Feb 3;7(2):e525. doi: 10.1038/bcj.2017.6.
Abstract
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.
摘要
原发性骨髓纤维化（PMF）是一种骨髓增殖性肿瘤，起源于造血干细胞的克隆性增殖，并导致进行性骨髓（BM）纤维化。虽然对参与PMF发生发展的细胞突变进行了大量研究，但值得注意的是细胞外基质（ECM）在BM纤维化进展中所起的重要作用。本文综述了PMF中ECM蛋白的作用，并强调了更好地理解BM微环境中ECM的调控如何可能带来PMF的联合治疗选择。
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