Extracellular matrix: The driving force of mammalian diseases.
Extracellular matrix: The driving force of mammalian diseases.
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DOI：
10.1016/j.matbio.2018.03.023
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发表时间：
2018-10
期刊：
影响因子：
--
通讯作者：
Gubbiotti MA
中科院分区：
文献类型：
--
作者：
Iozzo RV;Gubbiotti MA
就像莫扎特协奏曲的主旋律一样，巨大而普遍的细胞外基质驱动着每一个运动，并最终结束了交响乐，作为大多数（如果不是全部）随后发生的细胞内事件的基本调解人，它扮演着独特的角色。因此，几乎所有已知疾病的机制都可以追溯到基质的某个部分，通常以特定基质成分的异常量或活性水平的形式，这并不奇怪。这些缺陷显著影响下游信号传导轴，导致明显的细胞功能障碍、器官衰竭和死亡。从皮肤到骨骼，从血管到大脑，从眼睛到所有的内部器官，基质扮演着令人难以置信的角色，既是疾病的原因，也是逆转疾病的潜在手段。人类疾病包括结缔组织疾病、肌肉萎缩症、纤维化和癌症，都是细胞外基质驱动的疾病。理解和调节这些基质相关机制的能力可能会导致未来为这些患者发现新的治疗选择。
Like the major theme of a Mozart concerto, the immense and pervasive extracellular matrix drives each movement and ultimately closes the symphony, embracing a unique role as the fundamental mediator for most, if not all, ensuing intracellular events. As such, it comes as no surprise that the mechanism of just about every known disease can be traced back to some part of the matrix, typically in the form of an abnormal amount or activity level of a particular matrix component. These defects considerably affect downstream signaling axes leading to overt cellular dysfunction, organ failure, and death. From skin to bone, from vessels to brain, from eyes to all the internal organs, the matrix plays an incredible role as both a cause and potential means to reverse diseases. Human malaises including connective tissue disorders, muscular dystrophy, fibrosis, and cancer are all extracellular matrix-driven diseases. The ability to understand and modulate these matrix-related mechanisms may lead to the future discovery of novel therapeutic options for these patients.
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