多囊蛋白在骨形成及骨疾病治疗中的作用和机制

Role and mechanism of polycystin in bone formation and bone disease treatments

背景:多囊蛋白最早是在常染色体显性多囊肾病中发现的,它参与了囊肿的形成,后来又发现其与骨形成及颅缝早闭、骨质丢失和骨肉瘤等骨相关疾病的发生发展有关。目的:对多囊蛋白在骨形成及骨相关疾病的发生发展过程中所发挥的作用进行综述。方法:以“bone”or“bone formation mechanism”or“craniosynostosis”or“bone loss”or“osteosarcoma”and“polycystin”为检索词,检索PubMed数据库1990年1月至2020年4月的相关文献;同时以“多囊蛋白”and“骨”,“多囊蛋白”and“骨形成机制”,“多囊蛋白”and“骨质丢失”,“多囊蛋白”and“骨肉瘤”,“多囊蛋白”and“颅缝早闭”为检索词,检索CNKI数据库1979年1月至2020年4月的相关文献,最终选择45篇密切相关文献进行综述。结果与结论:①研究发现多囊蛋白作为机械感受器存在于骨细胞的质膜中,被外界机械刺激激活,并在细胞内触发信号通路,参与骨形成及骨丧失机制的调节,参与骨发育过程,并在颅缝早闭、骨质丢失和骨肉瘤等骨疾病的发生发展过程中起到重要作用;②迄今对多囊蛋白在颌面骨及颅骨疾病中的研究不多,对于多囊蛋白在骨骼的生理病理过程中的作用及调节机制尚待深入研究。

BACKGROUND:Polycystin was first discovered in autosomal dominant polycystic kidney disease,which was involved in the formation of cysts,and later it was found to be related to bone formation and the occurrence and development of bone-related diseases such as craniosynostosis,bone loss,and osteosarcoma.OBJECTIVE:To review the role of polycystin in bone formation mechanism and the occurrence and development of bone-related diseases.METHODS:PubMed database was searched for relevant documents from January 1990 to April 2020.The search terms were“bone”or“bone formation mechanism”or“craniosynostosis”or“bone loss”or“osteosarcoma”and“polycystin.”At the same time,CNKI database was searched for relevant documents from January 1979 to April 2020.The search terms were“polycystin”and“bone,”“polycystin”and“bone formation mechanism,”“polycystin”and“bone loss,”“polycystin”and“osteosarcoma,”“polycystin”and“craniosynostosis.”Finally,45 relevant documents were included for review.RESULTS AND CONCLUSION:Polycystin exists as a mechanoreceptor in the plasma membrane of bone cells,which is activated by external mechanical stimuli,and triggers signaling pathways in the cells,participates in the regulation of bone formation and bone loss mechanisms,participates in bone development,and plays an important role in the occurrence and development of bone diseases,such as craniosynostosis,bone loss,and osteosarcoma.To date,there have been few studies regarding the role of polycystin in maxillofacial bone and skull diseases,suggesting that in-depth studies on the role and mechanism of polycystin in the physiopathological processes of the skeletal bone are warranted.