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Abstract：
Transforming growth factor beta (TGFβ) is a multipotent cytokine that is sequestered in the extracellular matrix (ECM) through interactions with a number of ECM proteins. The ECM serves to concentrate latent TGFβ at sites of intended function, to influence the bioavailability and/or function of TGFβ activators, and perhaps to regulate the intrinsic performance of cell surface effectors of TGFβ signal propagation. The downstream consequences of TGFβ signaling cascades in turn provide feedback modulation of the ECM. This review covers recent examples of how genetic mutations in constituents of the ECM or TGFβ signaling cascade result in altered ECM homeostasis, cellular performance and ultimately disease, with an emphasis on emerging therapeutic strategies that seek to capitalize on this refined mechanistic understanding.
摘要：
转化生长因子β（TGFβ）是一种多能细胞因子，通过与许多ECM蛋白的相互作用而被隔离在细胞外基质（ECM）中。ECM用于将潜在的TGFβ浓缩在预期的功能位点，影响TGFβ激活剂的生物利用度和/或功能，并可能调节TGFβ信号传播的细胞表面效应子的内在性能。TGFβ信号传导级联的下游结果又提供ECM的反馈调制。这篇综述涵盖了ECM或TGFβ信号级联的成分中的基因突变如何导致ECM稳态改变的最新例子。细胞性能和最终的疾病，重点是寻求利用这种精致的机械理解的新兴治疗策略。
