Developmental and pathogenic mechanisms of basement membrane assembly.

Developmental and pathogenic mechanisms of basement membrane assembly.
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DOI:
10.2174/138161209787846766
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发表时间:
2009
影响因子:
3.1
通讯作者:
Patton BL
Patton BL
中科院分区:
医学4区
文献类型:
--
作者:
Yurchenco PD;Patton BL

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基底膜是片状细胞粘附的细胞外基质,充当细胞基质和固相激动剂,有助于组织组织、稳定性和分化。这些基质被组装成层粘连蛋白和 IV 型胶原蛋白的聚合物,这些胶原蛋白与巢蛋白和蛋白聚糖相连。它们与细胞表面分子结合,包括整合素和肌营养不良聚糖等信号转导受体,并与邻近的结缔组织形成附着物。细胞受体反过来提供基质和底层细胞骨架之间的联系。基底膜和相关成分(统称为基底膜区域)的遗传性疾病会破坏细胞外基质和/或其连接,从而影响神经、肌肉、皮肤、肾脏和其他组织。这些疾病可能是由于基质完整性、粘附强度和/或受体介导的信号传导丧失而引起的。了解基底膜区域组装的机制和由此产生的结构可以深入了解正常组织的发育和多种疾病的致病机制。
Basement membranes are sheet-like cell-adherent extracellular matrices that serve as cell substrata and solid-phase agonists, contributing to tissue organization, stability and differentiation. These matrices are assembled as polymers of laminins and type IV collagens that are tethered to nidogens and proteoglycans. They bind to cell surface molecules that include signal-transducing receptors such as the integrins and dystroglycan and form attachments to adjacent connective tissues. The cell receptors, in turn, provide links between the matrix and underlying cytoskeleton. Genetic diseases of basement membrane and associated components, collectively the basement membrane zone, disrupt the extracellular matrix and/or its linkages to affect nerve, muscle, skin, kidney and other tissues. These diseases can arise due to a loss of matrix integrity, adhesion strength and/or receptor-mediated signaling. An understanding of the mechanisms of basement membrane zone assembly and resulting structure can provide insights into the development of normal tissues and the pathogenic mechanisms that underlie diverse disorders.
层粘连蛋白和仿生细胞外弹性增强了乳腺上皮的功能分化。
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