Program in Developmental Therapeutics, Fox Chase Cancer Center, Philadelphia, PA 19111, USA.
Curr Opin Cell Biol. 2012 Oct;24(5):652-61. doi: 10.1016/j.ceb.2012.06.002. Epub 2012 Jul 19.
The primary cilium protrudes like an antenna from the cell surface, sensing mechanical and chemical cues provided in the cellular environment. In some tissue types, ciliary orientation to lumens allows response to fluid flow; in others, such as bone, ciliary protrusion into the extracellular matrix allows response to compression forces. The ciliary membrane contains receptors for Hedgehog, Wnt, Notch, and other potent growth factors, and in some instances also harbors integrin and cadherin family members, allowing receipt of a robust range of signals. A growing list of ciliopathies, arising from deficient formation or function of cilia, includes both developmental defects and chronic, progressive disorders such as polycystic kidney disease (PKD); changes in ciliary function have been proposed to support cancer progression. Recent findings have revealed extensive signaling dialog between cilia and extracellular matrix (ECM), with defects in cilia associated with fibrosis in multiple contexts. Further, a growing number of proteins have been determined to possess multiple roles in control of cilia and focal adhesion interactions with the ECM, further coordinating functionality. We summarize and discuss these recent findings.
纤毛从细胞表面像天线一样伸出，感知细胞环境中提供的机械和化学线索。在某些组织类型中，纤毛对管腔的定向允许对流体流动做出反应；在其他组织中，如骨骼，纤毛向细胞外基质的突出允许对压缩力做出反应。纤毛膜包含 Hedgehog、Wnt、Notch 和其他强效生长因子的受体，在某些情况下还含有整联蛋白和钙黏蛋白家族成员，允许接收广泛的信号。越来越多的纤毛病，源于纤毛的形成或功能缺陷，包括发育缺陷和慢性、进行性疾病，如多囊肾病 (PKD)；纤毛功能的改变被认为支持癌症的进展。最近的发现揭示了纤毛和细胞外基质 (ECM) 之间广泛的信号对话，纤毛缺陷与多种情况下的纤维化有关。此外，越来越多的蛋白质被确定具有控制纤毛和与 ECM 的焦点附着相互作用的多种作用，进一步协调功能。我们总结和讨论了这些最近的发现。
