文章：

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

原文发布日期：2017-02-03

DOI: 10.1038/bcj.2017.6

类型: Review

开放获取: 是

英文摘要：

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.

摘要翻译： 

原发性骨髓纤维化（PMF）是一种骨髓增殖性肿瘤，源于造血干细胞的克隆性增殖，并导致进行性骨髓纤维化。虽然PMF发展相关的细胞突变已得到广泛研究，但值得注意的是细胞外基质（ECM）在骨髓纤维化进展中发挥的重要作用。本综述探讨了参与PMF的ECM蛋白成分，并重点阐述了更好地理解BM微环境中ECM的调控机制如何可能为PMF带来联合治疗策略。

原文链接：

The role of the extracellular matrix in primary myelofibrosis