CAJ | 학술논문

视网膜色素变性(RP)是一种遗传性视网膜疾病,其典型的临床表现为早期视杆细胞的退行性病变以及视锥细胞胞体相对长期的存活.目前实验研究证实,通过腺相关病毒载体和慢病毒载体将微生物型的光敏感通道蛋白Channelrhodopsin-2或halorhodopsins导入RP模型鼠的视锥细胞胞体或其他细胞,可以使这些细胞获得光反应并激活视网膜传导通路,向视觉中枢传递视觉信息.因此,这为RP患者的复明带来了希望.
시망막색소변성(RP)시일충유전성시망막질병,기전형적림상표현위조기시간세포적퇴행성병변이급시추세포포체상대장기적존활.목전실험연구증실,통과선상관병독재체화만병독재체장미생물형적광민감통도단백Channelrhodopsin-2혹halorhodopsins도입RP모형서적시추세포포체혹기타세포,가이사저사세포획득광반응병격활시망막전도통로,향시각중추전체시각신식.인차,저위RP환자적복명대래료희망.
Retinitis pigmentosa (RP) is a hereditary disease characterized by the progressive loss of photoreceptor cells. Disease pathology primarily affects rod photoreceptor cells first but light-insensitive cone photoreceptor bodies can survivor longer. Delivered by adeno-associated viral vector or lentiviral vector, expression of microbial-type rhodopsin, channelrhodopsin-2 or archaebacterial halorhodopsin in the survived retinal cells can substitute for the native phototransduction cascade in mouse models of retinitis pigmentosa. It can also restore light sensitivity and activate all retinal cone pathways and drive sophisticated retinal circuit functions. Thus, expression of channelrhodopsins or halorhodopsin might be a potential strategy for the restoration vision of patients with RP.