Hedgehoge信号通路在多发性骨髓瘤中的作用

Roles of Hedgehog signaling pathway in multiple myeloma

近年来，随着硼替佐米、雷那度胺等新药和自体造血干细胞移植应用于多发性骨髓瘤（MM）的治疗，已使得MM患者预后得到显著改善，但是MM患者仍无法获得治愈。Hedgehog（Hh）信号通路调节胚胎发育与许多器官、组织中干细胞的增殖与分化。在对MM细胞系的研究及针对MM患者的临床试验中发现Hh信号通路存在异常激活，并且Hh信号通路在MM骨髓微环境及其他信号通路异常改变中起着主要作用。为了充分了解Hh信号通路在MM发病及治疗中的作用，进而为MM的治疗提供新的研究思路，笔者拟就Hh信号通路在MM骨髓微环境中的作用、与其他信号通路的联系及其相关药物治疗方面进行综述。

In recent years, with the application of new drugs, such as bortezomib and lenalidomide, and autologous hematopoietic stem cell transplantation, the prognosis of patients with multiple myeloma （MM） have significantly improved. However, MM still unable to be cured. The Hedgehog（Hh） pathway is required for cell-fate determination during the embryonic life, as well as cell growth and differentiation in the adult organism. The abnormal activation of Hh signaling pathway have been found in the study of MM cell lines and clinical trials. The Hh plays a significant role in the changes of bone marrow microenvirornent and the abnormal activation of other signaling pathway. To fully understand the function of Hh on pathogenesis and treatment of MM and bring forth new ideas to study MM, this review covers the role of Hh in bone marrow microenviroment, the interaction between Hh and other signaling pathway, and the drugs acting on Hh pathway.