| 付 瑶,贺 敬,孟宏学,姚国栋,耿敬姝.OFD1基因在初级纤毛及其与癌症发生发展方面的研究进展[J].现代生物医学进展英文版,2017,17(35):6980-6983. |
| OFD1基因在初级纤毛及其与癌症发生发展方面的研究进展 |
| Research Progress of OFD1 Gene in Primary Cilia and Cancer |
| Received:December 07, 2016 Revised:December 30, 2016 |
| DOI：10.13241/j.cnki.pmb.2017.35.042 |
| 中文关键词: OFD1基因 自噬 初级纤毛 癌症 |
| 英文关键词: OFD1 gene Autophagy Primary cilia Cancer |
| 基金项目:黑龙江省自然科学基金项目（LC2016038）；中国博士后基金项目（2015M581472）；黑龙江省博士后特别资助项目（LBH-TZ0616）；哈尔滨市科技创新人才专项奖金（孟宏学）；黑龙江省留学回国人员择优资助（孟宏学）；国家自然科学基金面上项目（81372785） |
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| 中文摘要: |
| 摘要：初级纤毛是由微管组成的细胞器，它负责感受细胞内环境并参与细胞间信号的转导。初级纤毛也是细胞信号整合的枢纽，是Hedgehog信号通路的关键分子，它的形成与细胞周期有关，初级纤毛的异常可以导致某些疾病的发生。研究表明人的乳腺癌和胰腺癌细胞中初级纤毛缺失，而在正常乳腺及胰腺细胞中呈激活状态，然而，初级纤毛在正常细胞和癌细胞之间的调控机制目前仍不清楚。OFD1是人类的一个致病基因，它是中心粒的末端组成成分，它负责调节中心粒的长度，同时也是初级纤毛形成所必需的基因。OFD1也定位于中心粒随体，研究表明自噬通过降解中心粒随体中的OFD1促进初级纤毛的形成，中心粒随体中OFD1基因的消除可以促进癌细胞中初级纤毛的恢复。因此，OFD1、初级纤毛和肿瘤之间存在一定联系，近期研究表明纤毛的缺失与肿瘤的细胞起源，遗传背景和肿瘤信号通路的损害关系密切，这将有助于我们对纤毛相关疾病的了解，有助于我们对包括癌症的发病和治疗方面的研究。 |
| 英文摘要: |
| ABSTRACT: Primary cilia, which are a microtubule-based organelle, are responsible for sensing the intracellular environment and participating in the transduction of intercellular signals. Primary cilia are also a hub for cellular signaling integration that are key molecules in the hedgehog pathways, which are associated with the cell cycle, and abnormalities in signal transduction pathways are commonly associated with several human diseases. Studies have shown that primary ciliogenesis is defective in human breast and pancre- atic cancer cells, but activated in the corresponding normal cells, however, regulation mechanism of primary cilia in between normal and cancer cells remains unclear. OFD1, a human disease gene, is a component of the distal ends of centrioles, which is responsible for reg- ulating the length of the centrioles, but also necessary for the formation of cilia. OFD1 also located in centriolar satellites, studies have shown that autophagy can promote the formation of cilia by degrading centriolar satellites in OFD1, removing of the OFD1 gene in the centriolar satellites may promote the recovery of cilia in cancer cells. Recent studies have indicated that the loss of cilia is closely related to the tumor. Thus, the relationship between OFD1, cilia and tumors is complex, including the origin of tumor cells, genetic background and signal damage. These mechanisms would help to understand the cilia-related diseases, including cancer and treatment. |
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